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Your search keyword '"Guiducci, Serena"' showing total 34 results
Start Over Author "Guiducci, Serena" Topic rheumatology
34 results on '"Guiducci, Serena"'

1. Adherence to therapy in people with rheumatic and musculoskeletal diseases in Italy and the role of the digital health: results of an expert Delphi consensus survey.

Author

Iagnocco A, Ciccia F, Conti F, D'Angelo S, Epis OM, Govoni M, Guiducci S, Iannone F, Mosca M, Salaffi F, Sebastiani GD, Sonnati M, and Caporali RF

Subjects
Humans, Consensus, Delphi Technique, Italy, Rheumatology, Rheumatic Diseases diagnosis, Rheumatic Diseases therapy, Musculoskeletal Diseases diagnosis, Musculoskeletal Diseases epidemiology, Musculoskeletal Diseases therapy
Abstract

Objectives: To present the results of a Delphi consensus survey among Italian rheumatologists on adherence to therapy in people with rheumatic and musculoskeletal diseases (RMDs) in Italy and the role of digital health., Methods: A taskforce of 12 rheumatologists comprehensively discussed the applicability of the 2020 EULAR Points to Consider (PtCs) for Italian rheumatology practice and formulated 44 new country-specific statements. Through an on-line survey, the panellists voted on their level of agreement with the statements using a 10-point Likert scale (0: no agreement; 10: total agreement). A combination of two distinct criteria, a mean agreement level ≥8 and a percentage of at least 75% of responses with a value ≥8, was deemed acceptable., Results: The consensus threshold was reached for 43 of the 44 country-specific statements. The following were acknowledged among the barriers to applicability of the recommendations: visit time too short; lack of resources; lack of a clear operational flow-chart; lack of communication skills and poor knowledge of techniques to improve patient adherence by healthcare professionals (HCPs)., Conclusions: This consensus initiative helps contribute to more widespread implementation of EULAR PtCs in Italian rheumatology practice. Optimisation of visit time, greater availability of resources, specific training, use of standardised and validated protocols, and active involvement of patients represent the main goals. Digital health can provide valuable support for the application of PtCs and, more generally, in improving adherence. A collaborative effort between HCPs, patients and their associations, scientific societies, and policymakers is strongly advocated to overcome some of the barriers.

Published
2023
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2. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology.

Author

De Angelis R, Ferri C, Giuggioli D, Bajocchi G, Dagna L, Bellando-Randone S, Zanframundo G, Foti R, Cacciapaglia F, Cuomo G, Ariani A, Rosato E, Lepri G, Girelli F, Riccieri V, Zanatta E, Bosello SL, Cavazzana I, Ingegnoli F, De Santis M, Murdaca G, Abignano G, Romeo N, Della Rossa A, Caminiti M, Iuliano AM, Ciano G, Beretta L, Bagnato G, Lubrano E, De Andres I, Giollo A, Saracco M, Agnes C, Cipolletta E, Lumetti F, Spinella A, Magnani L, Campochiaro C, De Luca G, Codullo V, Visalli E, Di Vico C, Gigante A, Pellagrino G, Pigatto E, Lazzaroni MG, Franceschini F, Generali E, Mennillo G, Barsotti S, Mariano GP, Furini F, Vultaggio L, Parisi S, Peroni CL, Rozza D, Zanetti A, Carrara G, Landolfi G, Scirè CA, Bianchi G, Fusaro E, Sebastiani GD, Govoni M, D'Angelo S, Cozzi F, Guiducci S, Doria A, Salvarani C, Iannone F, and Matucci-Cerinic M

Subjects
Humans, Seasons, Rheumatology, Scleroderma, Systemic, Autoimmune Diseases
Abstract

Objective: To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort., Methods: Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets., Results: Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001)., Conclusion: The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Published
2023
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3. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature.

Author

Ferri C, De Angelis R, Giuggioli D, Bajocchi G, Dagna L, Zanframundo G, Foti R, Cacciapaglia F, Cuomo G, Ariani A, Rosato E, Guiducci S, Girelli F, Riccieri V, Zanatta E, Bosello S, Cavazzana I, Ingegnoli F, De Santis M, Murdaca G, Abignano G, Romeo N, Della Rossa A, Caminiti M, Iuliano A, Ciano G, Beretta L, Bagnato G, Lubrano E, De Andres I, Giollo A, Saracco M, Agnes C, Lumetti F, Spinella A, Magnani L, Campochiaro C, De Luca G, Codullo V, Visalli E, Masini F, Gigante A, Bellando-Randone S, Pellegrino G, Pigatto E, Lazzaroni MG, Franceschini F, Generali E, Mennillo G, Barsotti S, Mariano GP, Calabrese F, Furini F, Vultaggio L, Parisi S, Peroni CL, Rozza D, Zanetti A, Carrara G, Landolfi G, Scirè CA, Bianchi G, Fusaro E, Sebastiani GD, Govoni M, D'Angelo S, Cozzi F, Doria A, Iannone F, Salvarani C, and Matucci-Cerinic M

Subjects
Antibodies, Antinuclear, Humans, Italy epidemiology, Phenotype, Registries, Tertiary Care Centers, Rheumatology, Scleroderma, Systemic diagnosis
Abstract

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature., Materials and Methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 ± 26.9 yrs.; mean disease duration 8.9 ± 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas., Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches., Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022. Published by Elsevier B.V.)

Published
2022
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4. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology.

Author

De Angelis R, Giuggioli D, Bajocchi G, Dagna L, Zanframundo G, Foti R, Cacciapaglia F, Cuomo G, Ariani A, Rosato E, Guiducci S, Girelli F, Riccieri V, Zanatta E, Bosello S, Cavazzana I, Ingegnoli F, Santis M, Murdaca G, Abignano G, Romeo N, Della Rossa A, Caminiti M, Iuliano A, Ciano G, Beretta L, Bagnato G, Lubrano E, De Andres I, Giollo A, Saracco M, Agnes C, Lumetti F, Spinella A, Magnani L, Campochiaro C, De Luca G, Codullo V, Visalli E, Masini F, Gigante A, Bellando-Randone S, Pellegrino G, Pigatto E, Dall'Ara F, Lazzaroni MG, Generali E, Mennillo G, Barsotti S, Mariano GP, Calabrese F, Furini F, Vultaggio L, Parisi S, Peroni CL, Risa AM, Rozza D, Zanetti A, Carrara G, Landolfi G, Scirè CA, Bianchi G, Fusaro E, Sebastiani GD, Govoni M, D'Angelo S, Cozzi F, Doria A, Iannone F, Salvarani C, Matucci-Cerinic M, and Ferri C

Subjects
Cross-Sectional Studies, Female, Humans, Italy epidemiology, Male, Registries, Sex Characteristics, Stroke Volume, Ventricular Function, Left, Rheumatology, Scleroderma, Systemic diagnosis, Sjogren's Syndrome
Abstract

Objective: There is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics., Methods: A multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared., Results: The overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs., Conclusion: Our study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex., (© 2022 by the Journal of Rheumatology.)

Published
2022
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5. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.

Author

van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Csuka ME, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, and Pope JE

Subjects
Consensus, Humans, Scleroderma, Systemic immunology, Sensitivity and Specificity, Diagnosis-Related Groups, Rheumatology, Scleroderma, Systemic classification, Scleroderma, Systemic diagnosis
Abstract

Objective: The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc., Methods: Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc., Results: It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc., Conclusion: The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease., (Copyright © 2013 by the American College of Rheumatology.)

Published
2013
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6. Systemic sclerosis: a critical digest of the recent literature.

Author

Tani C, Bellando Randone S, Guiducci S, and Della Rossa A

Subjects
Humans, Rheumatology methods, Rheumatology trends, Scleroderma, Systemic diagnosis, Scleroderma, Systemic etiology, Scleroderma, Systemic therapy
Abstract

Herewith we provide a critical digest of the recent literature on systemic sclerosis. The most relevant studies published over the last two years have been reviewed, with particular focus on the diagnosis, pathogenesis and treatment of the disease.

Published
2013

7. Recommendations for the use of biologic therapy in rheumatoid arthritis: update from the Italian Society for Rheumatology I. Efficacy.

Author

Caporali R, Conti F, Alivernini S, Atzeni F, Seriolo B, Cutolo M, Valesini G, Ferraccioli G, Sarzi-Puttini P, Salvarani C, Guiducci S, Zampogna G, Gremese E, Pipitone N, Scrivo R, Bugatti S, Montecucco C, and Matucci-Cerinic M

Subjects
Evidence-Based Medicine standards, Humans, Italy, Patient Selection, Treatment Outcome, Antirheumatic Agents therapeutic use, Arthritis, Rheumatoid therapy, Biological Products therapeutic use, Biological Therapy standards, Rheumatology standards, Societies, Medical standards
Abstract

Given the availability of novel biologic agents for the treatment of rheumatoid arthritis (RA), various national scientific societies have developed specific recommendations in order to assist rheumatologists in prescribing these drugs. The Italian Society for Rheumatology (Società Italiana di Reumatologia, SIR) decided to update its recommendations and, to this end, a systematic literature review was carried out and the evidence derived from it was discussed and summarised as expert opinions. Levels of evidence, strength of recommendations and levels of agreement were reported. The recommendations reported are intended to help prescribing rheumatologists to optimise the use of biologic agents in patients with RA seen in everyday practice; they are not to be considered as a regulatory rule.

Published
2011

8. Immunomodulatory properties of mesenchymal stem cells: a review based on an interdisciplinary meeting held at the Kennedy Institute of Rheumatology Division, London, UK, 31 October 2005.

Author

Tyndall A, Walker UA, Cope A, Dazzi F, De Bari C, Fibbe W, Guiducci S, Jones S, Jorgensen C, Le Blanc K, Luyten F, McGonagle D, Martin I, Bocelli-Tyndall C, Pennesi G, Pistoia V, Pitzalis C, Uccelli A, Wulffraat N, and Feldmann M

Subjects
Animals, Arthritis, Rheumatoid immunology, Arthritis, Rheumatoid surgery, Humans, Immunologic Factors immunology, London, Mesenchymal Stem Cell Transplantation methods, Mesenchymal Stem Cell Transplantation trends, Rheumatology trends, Congresses as Topic trends, Immunologic Factors therapeutic use, Mesenchymal Stem Cells immunology, Rheumatology methods
Abstract

Multipotent mesenchymal stromal cells isolated from bone marrow and other sites are currently being studied to determine their potential role in the pathogenesis and/or management of autoimmune diseases. In vitro studies have shown that they exhibit a dose-dependent antiproliferative effect on T and B lymphocytes, dendritic cells, natural killer cells and various B cell tumour lines--an effect that is both cell contact and soluble factor dependent. Animal models of autoimmune disease treated with multipotent mesenchymal stromal cells have mostly exhibited a positive clinical response, as have a limited number of patients suffering from acute graft versus host disease. This review summarizes the findings of a 1-day meeting devoted to the subject with the aim of coordinating efforts.

Published
2007
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9. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

Author

Hoffmann-Vold, Anna-Maria, Allanore, Yannick, Alves, Margarida, Brunborg, Cathrine, Airó, Paolo, Ananieva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P, Kowal-Bielecka, Otylia, Riccardi, Antonella, Distler, Oliver, Smith, Vanessa, and on behalf of the EUSTAR consortium, [ missing ]

Subjects
Male, Vital capacity, Databases, Factual, Vital Capacity, Severity of Illness Index, Pulmonary function testing, 0302 clinical medicine, QUALITY-OF-LIFE, Risk Factors, Pulmonary fibrosis, Prevalence, Immunology and Allergy, FIBROSIS, scleroderma, Prospective Studies, 610 Medicine & health, PREDICTORS, Lung, Interstitial lung disease, respiratory system, Middle Aged, Dysphagia, Europe, Performing Arts, Cohort, Disease Progression, Female, medicine.symptom, Adult, medicine.medical_specialty, Immunology, Genetics and Molecular Biology, Systemic Sclerosis, behavioral disciplines and activities, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, FEV1/FVC ratio, Rheumatology, Internal medicine, medicine, Humans, COHORT, autoimmune diseases, 030203 arthritis & rheumatology, DECLINE, Scleroderma, Systemic, pulmonary fibrosis, business.industry, MORTALITY, PULMONARY-FUNCTION, systemic, medicine.disease, respiratory tract diseases, body regions, 030228 respiratory system, General Biochemistry, EULAR SCLERODERMA TRIALS, business, Lung Diseases, Interstitial, FORCED VITAL CAPACITY
Abstract

ObjectivesTo identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up.MethodsEligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models.Results826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms.ConclusionSSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.

Published
2020

10. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the european scleroderma trials and research (eustar) cohort

Author

Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Wu, W., Jordan, S., Graf, N., de Oliveira Pena, J., Curram, J., Allanore, Y., Matucci-Cerinic, M., Pope, J. E., Denton, C. P., Khanna, D., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., Sarzi Puttini, P., Ege Üniversitesi, Chizzolini, Carlo, Allali, Danièle, University of Zurich, and Distler, Oliver

Subjects
INVOLVEMENT, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, Time Factors, Databases, Factual, Skin Diseases/etiology/mortality/physiopathology, PREDICTION, Fibrosi, 2745 Rheumatology, Diffuse/complications/mortality/pathology, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, Kaplan-Meier Estimate, ddc:616.07, Severity of Illness Index, Scleroderma, Cohort Studies, PROGNOSTIC-FACTORS, Fibrosis, Medicine and Health Sciences, scleroderma, Lung, Skin, integumentary system, progressive skin fibrosis, Lung function decline, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, DEATH, Middle Aged, ddc, Europe, VARIABILITY, factual, Cohort, Visceral organ progression, 2723 Immunology and Allergy, Disease Progression, Female, Survival Analysi, Life Sciences & Biomedicine, Cohort study, Human, Adult, Skin/pathology, medicine.medical_specialty, databases, All-cause death, risk analysis, diffuse cutaneous systemic sclerosis, 610 Medicine & health, IMPROVEMENT, Systemic Sclerosis, Skin Diseases, THICKNESS SCORE, VALIDATION, Databases, FEV1/FVC ratio, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, Humans, Factual, Survival analysis, 2403 Immunology, Science & Technology, Proportional hazards model, business.industry, Surrogate endpoint, MORTALITY, Skin Disease, fibrosis, Progressive skin fibrosi, Lung/physiopathology, biomarkers, Diffuse cutaneous systemic sclerosi, medicine.disease, Survival Analysis, all-cause death, lung function decline, visceral organ progression, adult, cohort studies, databases, factual, disease progression, female, humans, Scleroderma, Diffuse, Cohort Studie, business
Abstract

PubMed: 30852552, Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ?7, valid mRSS at 12±3 months after baseline and ?1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ?25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ?10% (53.6% vs 34.4%; p, Bayer Bayer, 1Department of Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Clinical Development Pulmonology, Bayer Us llC, Whippany, new Jersey, Usa 4Data science and analytics, Bayer plc, Reading, UK 5Rheumatology a Department, Paris Descartes University, inseRM U1016, sorbonne, Paris Cité, Cochin Hospital, Paris, France 6Division of Rheumatology, University of Florence, Florence, italy 7Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Western Ontario, Canada 8Department of Rheumatology, Royal Free Hospital, University College london, london, UK 9scleroderma Program, Department of internal Medicine, Division of Rheumatology, University of Michigan, ann arbor, Michigan, Usa Acknowledgements The authors thank nicole schneider for excellent administration and data entry into the eUsTaR cohort. Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., This study was supported by a grant from Bayer aG.

Published
2019

11. Phenotypes determined by cluster analysis and their survival in the prospective European Scleroderma Trials and Research cohort of patients with systemic sclerosis

Author

Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E, Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J, Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P, Denisov, Lev N, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D, Yoshinari, Natalino H, Marangoni, Roberta G, Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C, Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A, Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, Poindron, Vincent, EUSTAR Collaborators, Guiducci, S., Walker, U., Kyburz, D., Lapadula, G., Iannone, F., Maurer, B., Jordan, S., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Siegert, E., Rednic, S., Nicoara, I., Kahan, A., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Stork, J., Inanc, M., Carreira, P.E., Novak, S., Czirják, L., Varju, C., Chizzolini, C., Kucharz, E.J., Kotulska, A., Kopec-Medrek, M., Widuchowska, M., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Wu, C., Marjanovic, Z., Faivre, H., Hij, D., Dhamadi, R., Airò, P., Hesselstrand, R., Wollheim, F., Wuttge, D.M., Andréasson, K., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Trotta, F., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Mauriziano, O., Maria Bambara, L., Caramaschi, P., Morovic-Vergles, J., Black, C., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Burkhardt, H., Himsel, A., Bajocchi, G., Maria Nuova, A.S., João Salvador, M., Pereira Da Silva, J.A., Stamenkovic, B., Stankovic, A., Francesco Selmi, C., De Santis, M., Marasini, B., Tikly, M., Ananieva, L.P., Denisov, L.N., Müller-Ladner, U., Frerix, M., Tarner, I., Scorza, R., Puppo, F., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szücs, G., Szamosi, S., Zea Mendoza, A., de la Puente, C., Sifuentes Giraldo, W.A., Midtvedt, Ø., Reiseter, S., Garen, T., Valesini, G., Riccieri, V., Maria Ionescu, R., Opris, D., Groseanu, L., Wigley, F.M., Sfrent Cornateanu, R., Ionitescu, R., Maria Gherghe, A., Soare, A., Gorga, M., Bojinca, M., Mihai, C., Milicescu, M., Sunderkötter, C., Kuhn, A., Sandorfi, N., Schett, G., Distler, J.H., Beyer, C., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Paolo Cantatore, F., Corrado, A., Ullman, S., Iversen, L., Alberto von Mühlen, C., Marilu Bohn, J., Scussel Lonzetti, L., Rosa Pozzi, M., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Houssiau, F.A., Jose Alegre-Sancho, J., Krummel-Lorenz, B., Saar, P., Aringer, M., Günther, C., Westhovens, R., de Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Üprus, M., Otsa, K., Yavuz, S., Granel, B., Cezar Radominski, S., de Souza Müller, C., Azevedo, V.F., Jimenez, S., Busquets, J., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Popa, S., Zenone, T., Pileckyte, M., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Sampaio-Barros, P.D., Yoshinari, N.H., Marangoni, R.G., Martin, P., Fuocco, L., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Abdel Atty Mohamed, W.A., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C.M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Chirieac, R., Ancuta, C., Furst, D.E., Villiger, P., Adler, S., van Laar, J., Kayser, C., Eduardo C, A.L., Fathi, N., Hassanien, M., de la Peña Lefebvre, P.G., Rodriguez Rubio, S., Valero Exposito, M., Sibilia, J., Chatelus, E., Gottenberg, J.E., Chifflot, H., Litinsky, I., Emery, P., Buch, M., Del Galdo, F., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Ann Saketkoo, L., Lasky, J.A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Luca Brucato, A., Lupi, E., Rosner, I., Rozenbaum, M., Slobodin, G., Boulman, N., Rimar, D., Couto, M., Spertini, F., Ribi, C., Buss, G., Kahl, S., Hsu, V.M., Chen, F., McCloskey, D., Malveaux, H., Louis Pasquali, J., Martin, T., Gorse, A., Guffroy, A., Poindron, V., and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, Databases, Factual, systemic sclerosis, SUBSETS, Disease, Severity of Illness Index, Scleroderma, DISEASE, 0302 clinical medicine, Medicine and Health Sciences, Immunology and Allergy, Cluster Analysis, CRITERIA, Prospective Studies, Prospective cohort study, skin and connective tissue diseases, integumentary system, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, Adult, Aged, Autoantibodies/blood, Europe/epidemiology, Female, Humans, Middle Aged, Phenotype, Prognosis, Scleroderma, Diffuse/blood, Scleroderma, Diffuse/epidemiology, Scleroderma, Diffuse/pathology, Scleroderma, Limited/blood, Scleroderma, Limited/epidemiology, Scleroderma, Limited/pathology, Scleroderma, Systemic/blood, Scleroderma, Systemic/epidemiology, Scleroderma, Systemic/pathology, Connective tissue disease, ddc, Europe, MANIFESTATIONS, Cohort, Life Sciences & Biomedicine, medicine.medical_specialty, Immunology, PROFILE, CLASSIFICATION, 03 medical and health sciences, Rheumatology, Scleroderma, Limited, Internal medicine, Severity of illness, medicine, Autoantibodies, 030203 arthritis & rheumatology, Science & Technology, Scleroderma, Systemic, business.industry, Autoantibody, Systemic sclerosis (SSc), medicine.disease, 030104 developmental biology, Scleroderma, Diffuse, business
Abstract

OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. RESULTS: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. CONCLUSION: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published

Published
2019

12. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: A European Scleroderma Trials and Research (EUSTAR) analysis

Author

Becker, Mike, Graf, Nicole, Sauter, Rafael, Allanore, Yannick, Curram, John, Denton, Christopher P., Khanna, Dinesh, Matucci-Cerinic, Marco, Pena, Janethe de Oliveira, Pope, Janet E., Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Ege Üniversitesi, University of Zurich, Distler, Oliver, Chizzolini, Carlo, Allai, Daniela, Becker, M., Graf, N., Sauter, R., Allanore, Y., Curram, J., Denton, C. P., Khanna, D., Matucci-Cerinic, M., de Oliveira Pena, J., Pope, J. E., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., and Sarzi Puttini, P.

Subjects
INVOLVEMENT, SELECTION, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, predictive factors, systemic sclerosis, 2745 Rheumatology, epidemiologic methods, morbidity, Disease, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, INTERSTITIAL LUNG-DISEASE, disease worsening, mortality, predictive factors, systemic sclerosis, predictive factor, disease worsening, DESIGN, middle aged, Medicine and Health Sciences, FIBROSIS, scleroderma, SKIN THICKNESS SCORE, mortality, skin and connective tissue diseases, Prospective cohort study, humans, lung diseases, ddc:616, education.field_of_study, heart diseases, integumentary system, clinical trials as topic, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, Interstitial lung disease, follow-up studies, ddc, female, Cohort, 2723 Immunology and Allergy, europe, Life Sciences & Biomedicine, CLINICAL-TRIALS, survival rate, medicine.medical_specialty, Population, 610 Medicine & health, disease progression, male, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, REGRESSION, medicine, severity of illness index, education, Survival rate, METAANALYSIS, 2403 Immunology, Science & Technology, Scleroderma, Systemic, business.industry, MORTALITY, systemic, medicine.disease, prospective studies, Clinical trial, prognosis, scleroderma, diffuse, scleroderma, systemic, Scleroderma, Diffuse, business
Abstract

PubMed: 31227488, Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12±3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials. © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ., Bayer Bayer, United Kingdom Université Paris Descartes Li Ka Shing Foundation, LKSF University of Michigan, U-M, 1Department of Rheumatology and the Centre of experimental Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Big Data institute, li Ka shing Centre for Health information and Discovery, nuffield Department of Medicine, University of Oxford, Oxford, UK 4Rheumatology a Department, Paris Descartes University, sorbonne Paris Cité, Cochin Hospital, Paris, France 5Data science and analytics, Bayer plc, Reading, UK 6UCl Division of Medicine, Royal Free Campus, london, UK 7Division of Rheumatology, Department of internal Medicine, University of Michigan scleroderma Program, University of Michigan, ann arbor, Michigan, Usa 8Department of experimental and Clinical Medicine, University of Florence, Florence, italy 9Bayer Us llC, Whippany, new Jersey, Usa 10Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Ontario, Canada Acknowledgements The R-code for the linear Mi-lassO was received from Qixuan Chen.21 Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., Contributors study conception and design, acquisition of data, analysis and interpretation of data and drafting and revising the article: OD and MB; analysis and interpretation of data: OD, MB, Rs and nG. all authors have critically reviewed and approved the final submitted version to be published. Funding This study was supported by a grant from Bayer aG. Bayer employees are coauthors of this paper and supported the study design and interpretation of the data, but otherwise Bayer had no influence on the study., Competing interests MOB declares no conflict of interest. OD has had consultancy relationships with actelion, Bayer, Biogen idec, Boehringer ingelheim, Chemomab, espeRare foundation, Genentech/Roche, GsK, inventiva, italfarmaco, lilly, medac, Medimmune, Mitsubishi Tanabe Pharma, Pharmacyclics, novartis, Pfizer, sanofi, sinoxa and UCB in the area of potential treatments of scleroderma and its complications. OD has received research funding from actelion, Bayer, Boehringer ingelheim, Mitsubishi Tanabe Pharma and Roche in the area of potential treatments of scleroderma and its complications. OD has a patent for mir-29 licensed for the treatment of systemic sclerosis. DK has consultancy relationships and/or has received grant/research support from Bayer, Bristol-Myers squibb, Boehringer ingelheim, Genentech/Roche, niH, Pfizer, sanofi-aventis Pharmaceuticals, actelion Pharmaceuticals Us, Chemomab, Corbus, Covis, Cytori, eicos, eMD serono, Gilead, GlaxosmithKline, and UCB Pharma. He is a shareholder of eicos. CPD has consultancy relationships with and/or has received speakers’ bureau fees from actelion Pharmaceuticals Us, Bayer aG, GlaxosmithKline, Csl Behring, Merck serono, Roche Pharmaceuticals, Genentech and Biogen iDeC inc., inventiva, sanofi-aventis Pharmaceuticals and Boehringer ingelheim. JeP has consultancy relationships with and/or has received grant/research support from actelion, Bayer aG, Bristol-Myers squibb, Merck, Pfizer inc. and Roche. MM-C has consultancy relationships and/ or has received grant/research support from Pfizer, Bristol-Myers squibb, actelion, UCB Pharma, Bayer, Chemomab, Genentech/Roche, inventiva and lilly. Ya has consultancy relationships with and/or has received grant/research support from actelion, Pharmaceuticals Us, Bayer aG, Bristol-Myers squibb, inventiva, Medac, Pfizer inc., Roche Pharmaceuticals, Genentech and Biogen iDeC inc., sanofi-aventis Pharmaceuticals and servier. JdOP and JC are employees of Bayer. nTG has nothing to disclose.

Published
2019

13. Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Author

Blagojevic, Jelena, Bellando-Randone, Silvia, Abignano, Giuseppina, Avouac, Jérôme, Cometi, L, Czirják, László, Denton, Christopher P, Distler, Oliver, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A, Allanore, Yannick, Müller-Ladner, Ulf, Del Galdo, Francesco, Matucci-Cerinic, Marco, EUSTAR co-workers, University of Zurich, Blagojevic, Jelena, Blagojevic, J., Bellando-Randone, S., Abignano, G., Avouac, J., Cometi, L., Czirják, L., Denton, C. P., Distler, O., Frerix, M., Guiducci, S., Huscher, D., Jaeger, V. K., Lóránd, V., Maurer, B., Nihtyanova, S., Riemekasten, G., Siegert, E., Tarner, I. H., Vettori, S., Walker, U. A., Allanore, Y., Müller-Ladner, U., Del Galdo, F., and Matucci-Cerinic, M.

Subjects
0301 basic medicine, Male, lcsh:Diseases of the musculoskeletal system, Observational Trial, 2745 Rheumatology, Digital ulcer, Categorisation, Scleroderma, Systemic sclerosi, 0302 clinical medicine, Surveys and Questionnaires, Immunology and Allergy, Prospective Studies, 10051 Rheumatology Clinic and Institute of Physical Medicine, Digital ulcers, Middle Aged, Calcium Channel Blockers, Classification, 3. Good health, Clinical Practice, Categorization, 2723 Immunology and Allergy, Systemic sclerosis, Drug Therapy, Combination, Female, Research Article, Adult, medicine.medical_specialty, Immunology, 610 Medicine & health, Sildenafil Citrate, Fingers, 03 medical and health sciences, Rheumatology, Skin Ulcer, medicine, Humans, In patient, European Union, Iloprost, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Bosentan, Essential item, medicine.disease, 030104 developmental biology, Essential items, Physical therapy, Observational study, lcsh:RC925-935, business
Abstract

Background: A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc).Methods: DeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked.Results: A total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU.Conclusions: For clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated while further implementation of DU classification and categorisation is warranted.Trial registration: Observational trial on DU (OT1) is one of the five trials of the DeSScipher project (ClinicalTrials.gov; OT1 Identifier: NCT01836263, posted on April 19, 2013).

Published
2018

14. Digital ulcers predict a worse disease course in patients with systemic sclerosis

Author

Mihai, Carina, Landewé, Robert, Van Der Heijde, Désirée, Walker, Ulrich A., Constantin, Paul I., Gherghe, Ana Maria, Ionescu, Ruxandra, Rednic, Simona, Allanore, Yannick, Avouac, Jéroˆme, Czirják, László, Hachulla, Eric, Riemekasten, Gabriela, Cozzi, Franco, Airò, Paolo, Cutolo, Maurizio, Mueller Ladner, Ulf, Matucci Cerinic, Marco, Launay, David, Dobrota, Rucsandra, Sfrent Cornateanu, Roxana, Zingarelli, Stefania, Pigatto, Erika, Cuomo, Giovanna, Caramaschi, Paola, Ananieva, Lidia, Ullman, Susanne, Iversen, Line, Gurman, Alexandra Balbir, Braun Moscovici, Yolanda, Carreira, Patricia E., Joven, Beatriz E., Minier, Tünde, Guiducci, Serena, Bellando Randone, Silvia, Pellerito, Raffaele, Hunzelmann, Nicolas, Tarner, Ingo H., Radominski, Sebastião Cezar, De Souza Müller, Carolina, Iannone, Florenzo, Henes, Jörg, Bancel, Dominique Farge, Damjanov, Nemanja, Ostojic, Predrag, Pozzi, Maria Rosa, Hesselstrand, Roger, Denton, Christopher, Krasowska, Dorota, Tikly, Mohammed, Riccieri, Valeria, Cantatore, Francesco Paolo, Corrado, Ada, Da Silva, José Antonio Pereira, Salvador, Maria João, Tyndall, Alan, Gabrielli, Armando, Distler, Oliver, Jordan, Suzan, Heitmann, Stefan, Burkhardt, Harald, Himsel, Andrea, Rozman, Blaz, Smith, Vanessa, Keyser, Filip De, Kalitena, Dusanka Martinovic, Radic, Mislav, Filipescu, Ileana, Petcu, Ana, Vlachoyiannopoulos, Panayiotis, Kucharz, Eugene J., Widuchowska, Malgorzata, Kopec Medrek, Magdalena, Kotulska, Anna, Szücs, Gabriella, Stankovic, Aleksandra, Stamenkovic, Bojana, Selmi, Carlo Francesco, Santis, Maria De, Marasini, Bianca, Coleiro, Bernard, Santamaria, Vera Ortiz, Westhovens, René, Becvár, Radim, Novak, Srdan, Engelhart, Merete, Meroni, Pierluigi, Ingegnoli, Francesca, Zeni, Silvana, Sulli, Alberto, Distler, Jörg, Yavuz, Sule, Montecucco, Carlomaurizio, Eyerich, Kilian, Krummel Lorenz, Brigitte, Zenone, Thierry, Midtvedt, Øyvind, Chizzolini, Carlo, Seidel, Matthias, Oleszowsky, Mara, Üprus, Maria, Opriş, Daniela, Groseanu, Laura, Bielecka, Otylia Kowal, Antonio, Zea Mendoza, Szechinski, Jacek, Morovic Vergles, Jadranka, Scorza, Raffaella, Puppo, Francesco, Mathieu, Alessandro, Anic, Branimir, Stork, Jiri, Stebbings, Simon, Inanc, Murat, Hasler, Paul, Von Mühlen, Carlos Alberto, Aringer, Martin, Popa, Sergei, Mengtao, Li, Rosato, Edoardo, University of Zurich, and Mihai, Carina

Subjects
Genetics and Molecular Biology (all), Lung Diseases, Male, 0301 basic medicine, Databases, Factual, Epidemiology, 2745 Rheumatology, Vital Capacity, Disease, Biochemistry, Scleroderma, Systemic autoimmune disease, High morbidity, 0302 clinical medicine, Cardiovascular Disease, Immunology and Allergy, Medicine (all), 10051 Rheumatology Clinic and Institute of Physical Medicine, Systemic Sclerosis, Adult, Aged, Cardiovascular Diseases, Disease Progression, Female, Hand Dermatoses, Humans, Hypertension, Pulmonary, Kidney Diseases, Middle Aged, Multivariate Analysis, Prognosis, Proportional Hazards Models, Pulmonary Diffusing Capacity, Retrospective Studies, Scleroderma, Systemic, Skin Ulcer, Stroke Volume, Fingers, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Orvostudományok, Pulmonary, Hypertension, 2723 Immunology and Allergy, medicine.medical_specialty, 610 Medicine & health, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, Disease course, Databases, 03 medical and health sciences, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Cox proportional hazards regression, medicine, In patient, Factual, 030203 arthritis & rheumatology, 2403 Immunology, business.industry, Systemic, medicine.disease, 030104 developmental biology, Physical therapy, business
Abstract

ObjectiveSystemic sclerosis (SSc) is a systemic autoimmune disease with high morbidity and significant mortality. There is a great need of predictors that would allow risk stratification of patients with SSc and ultimately initiation of treatment early enough to ensure optimal clinical results. In this study, we evaluated whether a history of digital ulcers (HDU) at presentation may be a predictor of vascular outcomes and of overall clinical worsening and death in patients with SSc.MethodsPatients from the EULAR Scleroderma Trials and Research (EUSTAR) database, satisfying at inclusion the 1980 American College of Rheumatology classification criteria for SSc, who had a follow-up of at least 3 years since baseline or who have died, were included in the analysis. HDU at presentation as a predictor of disease worsening or death was evaluated by Cox proportional hazards regression analysis.Results3196 patients matched the inclusion criteria (male sex 13.2%, 33.4% diffuse subset). At presentation, 1092/3196 patients had an HDU (34.1%). In multivariable analysis adjusting for age, gender and all parameters considered potentially significant, HDU was predictive for the presence of active digital ulcers (DUs) at prospective visits (HR (95% CI)): 2.41 (1.91 to 3.03), pConclusionsIn patients with SSc, HDU at presentation predicts the occurrence of DUs at follow-up and is associated with cardiovascular worsening and decreased survival.

Published
2015

15. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: A longitudinal EUSTAR study

Author

Jaeger, Veronika K., Wirz, Elina G., Allanore, Yannick, Rossbach, Philipp, Riemekasten, Gabriela, Hachulla, Eric, Distler, Oliver, Airò, Paolo, Carreira, Patricia E., Gurman, Alexandra Balbir, Tikly, Mohammed, Vettori, Serena, Damjanov, Nemanja, Müller Ladner, Ulf, Distler, Jörg H. W., Mangtao, Li, Walker, Ulrich A., Ananieva, Lidia, Heitmann, Stefan, Rednic, Simona, Riccieri, Valeria, Szmyrka Kaczmarek, Magdalena, Farge, Dominique, Lapadula, Giovanni, Matucci Cerinic, Marco, Guiducci, Serena, Hunzelmann, Nicolas, Ricci, Massimo, Mihai, Carina, Veale, Douglas, Hesselstrand, Roger, Mariok, Eduardo, Smith, Vanessa, Tarner, Ingo H., Kucharz, Eugene J., Czirják, László, Martinovic, Duska, Solanki, Kamal, Ancuta, Codrina Mihaela, Sibilia, Jean, Paola, Caramaschi, Hassanien, Manal, Kahl, Sarah, Wigley, Frederick, Vanthuyne, Marie, Opris, Daniela, Radominski, Sebastião C., Monaco, Andrea Lo, Corrado, Ada, Koehm, Michaela, Codullo, Veronica, Radim, Bevcar, Loyo, Esthela, Üprus, Maria, Pellerito, Raffaele, Zenone, Thierry, Gabrielli, Armando, Kowal Bielecka, Otylia, Rozman, Blaz, Scorza, Raffaella, Saketkoo, Lesley Ann, Midtvedt, Oyvind, Von Mühlen, Carlos Alberto, Henes, Jörg, Branimir, Ani, Hasler, Paul, Yavuz, Sule, Adler, Sabine, Krummel Lorenz, Brigitte, Posa, Magdalena, Engelhart, Merete, Denton, Christopher, Krasowska, Dorota, Lefebvre, Paloma Garcia De La Peña, Cozzi, Franco, Mouthon, Luc, Rosato, Edoardo, Selmi, Carlo, Sancho, Juan José Alegre, Mallia, Carmel, Limonta, Massimiliano, Seidel, Matthias, Foti, Rosario, Stamp, Lisa, Ullman, Susanne, Stebbings, Simon, Santamaria, Vera Ortiz, Del Galdo, Francesco, De Langhe, Ellen, Mathieu, Alessandro, Sunderkötter, Cord, Eyerich, Kilian, Stamenkovic, Bojana, Novak, Srdan, Sampaio Barros, Percival D., Kayser, Cristiane, Litinsky, Ira, Couto, Maura, Jaeger, Veronika K., Wirz, Elina G., Allanore, Yannick, Rossbach, Philipp, Riemekasten, Gabriela, Hachulla, Eric, Distler, Oliver, Airã², Paolo, Carreira, Patricia E., Gurman, Alexandra Balbir, Tikly, Mohammed, Vettori, Serena, Damjanov, Nemanja, Mã¼ller ladner, Ulf, Distler, Jã¶rg H. W., Li, Mangtao, Walker, Ulrich A., Ananieva, Lidia, Heitmann, Stefan, Rednic, Simona, Riccieri, Valeria, Szmyrka kaczmarek, Magdalena, Farge, Dominique, Lapadula, Giovanni, Matucci cerinic, Marco, Guiducci, Serena, Hunzelmann, Nicola, Ricci, Massimo, Mihai, Carina, Veale, Dougla, Hesselstrand, Roger, Mariok, Eduardo, Smith, Vanessa, Tarner, Ingo H., Kucharz, Eugene J., Czirjã¡k, Lã¡szlã³, Martinovic, Duska, Solanki, Kamal, Ancuta, Codrina Mihaela, Sibilia, Jean, Paola, Caramaschi, Hassanien, Manal, Kahl, Sarah, Wigley, Frederick, Vanthuyne, Marie, Opris, Daniela, Radominski, Sebastiã£o C., Monaco, Andrea Lo, Corrado, Ada, Koehm, Michaela, Codullo, Veronica, Radim, Bevcar, Loyo, Esthela, Ãœprus, Maria, Pellerito, Raffaele, Zenone, Thierry, Gabrielli, Armando, Kowal bielecka, Otylia, Rozman, Blaz, Scorza, Raffaella, Saketkoo, Lesley Ann, Midtvedt, Oyvind, Von Mã¼hlen, Carlos Alberto, Henes, Jã¶rg, Branimir, Ani, Hasler, Paul, Yavuz, Sule, Adler, Sabine, Krummel lorenz, Brigitte, Posa, Magdalena, Engelhart, Merete, Denton, Christopher, Krasowska, Dorota, Lefebvre, Paloma Garcia De La Peã±a, Cozzi, Franco, Mouthon, Luc, Rosato, Edoardo, Selmi, Carlo, Sancho, Juan Josã© Alegre, Mallia, Carmel, Limonta, Massimiliano, Seidel, Matthia, Foti, Rosario, Stamp, Lisa, Ullman, Susanne, Stebbings, Simon, Santamaria, Vera Ortiz, Del Galdo, Francesco, De Langhe, Ellen, Mathieu, Alessandro, Sunderkã¶tter, Cord, Eyerich, Kilian, Stamenkovic, Bojana, Novak, Srdan, Sampaio barros, Percival D., Kayser, Cristiane, Litinsky, Ira, Couto, Maura, and University of Zurich

Subjects
Male, Genetics and Molecular Biology (all), Pulmonology, Blood Pressure, Vascular Medicine, Biochemistry, Scleroderma, Diagnostic Radiology, 0302 clinical medicine, Retrospective Studie, Longitudinal Studies, lcsh:Science, integumentary system, Incidence, Arteries, Systolic Pressure, 3. Good health, Echocardiography, Cohort, Regression Analysis, Human, medicine.medical_specialty, Imaging Techniques, Gastrointestinal Disease, Immunology, 1100 General Agricultural and Biological Sciences, Skin Diseases, 03 medical and health sciences, FEV1/FVC ratio, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Humans, Autoantibodies, Retrospective Studies, Aged, 1000 Multidisciplinary, Biochemistry, Genetics and Molecular Biology (all), Proportional hazards model, lcsh:R, Biology and Life Sciences, Proteins, medicine.disease, Agricultural and Biological Sciences (all), Blood Vessels, lcsh:Q, Clinical Medicine, Ejection Fraction, Lung Diseases, Kidney Disease, Physiology, Gastrointestinal Diseases, lcsh:Medicine, Longitudinal Studie, Kaplan-Meier Estimate, Medicine (all), Lung Disease, Pericardial effusion, Risk Factors, Medizinische Fakultät, Immune Physiology, Ultrasound Imaging, Medicine and Health Sciences, 030212 general & internal medicine, Pulmonary Arteries, Connective Tissue Diseases, Immune System Proteins, Pulmonary Hypertension, Multidisciplinary, Ejection fraction, Radiology and Imaging, Incidence (epidemiology), 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Autoantibodie, Heart Disease, Kidney Diseases, Female, Anatomy, Research Article, Adult, Heart Diseases, Cardiology, 610 Medicine & health, Research and Analysis Methods, Antibodies, Regression Analysi, Autoimmune Diseases, Diagnostic Medicine, Internal medicine, medicine, ddc:610, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, Risk Factor, Skin Disease, Raynaud Disease, Retrospective cohort study, Surgery, Cardiovascular Anatomy, Clinical Immunology, business
Abstract

Objective Systemic sclerosis (SSc) is a rare and clinically heterogeneous autoimmune disorder characterised by fibrosis and microvascular obliteration of the skin and internal organs. Organ involvement mostly manifests after a variable period of the onset of Raynaud's phenomenon (RP). We aimed to map the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI) and renal involvement in the early course of SSc. Methods In the EUSTAR cohort, patients with early SSc were identified as those who had a visit within the first year after RP onset. Incident SSc organ manifestations and their risk factors were assessed using Kaplan-Meier methods and Cox regression analysis. Results Of the 695 SSc patients who had a baseline visit within 1 year after RP onset, the incident non-RP manifestations (in order of frequency) were: skin sclerosis (75%) GI symptoms (71%), impaired diffusing capacity for monoxide40mmHg (14%), and renal crisis (3%). In the heart, incidence rates were highest for diastolic dysfunction, followed by conduction blocks and pericardial effusion. While the main baseline risk factor for a short timespan to develop FVC impairment was diffuse skin involvement, for PAPsys>40mmHg it was higher patient age. The main risk factors for incident cardiac manifestations were anti-topoisomerase autoantibody positivity and older age. Male sex, anti-RNA-polymerase-III positivity, and older age were risk factors associated with incident renal crisis. Conclusion In SSc patients presenting early after RP onset, approximately half of all incident organ manifestations occur within 2 years and have a simultaneous rather than a sequential onset. These findings have implications for the design of new diagnostic and therapeutic strategies aimed to ‘widen' the still very narrow ‘window of opportunity'. They may also enable physicians to counsel and manage patients presenting early in the course of SSc more accurately.

Published
2016

16. Update of EULAR recommendations for the treatment of systemic sclerosis.

Author

Kowal-Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Jörg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, and Hunzelmann, Nicolas

Subjects
ACE inhibitors, FLUOXETINE, GASTROINTESTINAL disease treatment, THERAPEUTIC use of glucocorticoids, KIDNEY disease treatments, LUNG disease treatment, PULMONARY hypertension treatment, ULCER treatment, HETEROCYCLIC compounds, SEROTONIN uptake inhibitors, PHOSPHODIESTERASE inhibitors, PROSTAGLANDINS I, DELPHI method, ENDOTHELINS, FINGERS, GASTROINTESTINAL diseases, HEMATOPOIETIC stem cell transplantation, KIDNEY diseases, LUNG diseases, PULMONARY hypertension, RAYNAUD'S disease, RHEUMATOLOGY, SYSTEMIC scleroderma, ULCERS, CHEMICAL inhibitors, DISEASE complications, THERAPEUTICS
Abstract

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc. [ABSTRACT FROM AUTHOR]

Published
2017
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17. Digital ulcers in scleroderma: staging, characteristics and sub-setting through observation of 1614 digital lesions.

Author

Amanzi, Laura, Braschi, Francesca, Fiori, Ginevra, Galluccio, Felice, Miniati, Irene, Guiducci, Serena, Conforti, Maria-Letizia, Kaloudi, Olga, Nacci, Francesca, Sacu, Oana, Candelieri, Antonio, Pignone, Alberto, Rasero, Laura, Conforti, Domenico, and Matucci-Cerinic, Marco

Subjects
SYSTEMIC scleroderma, GANGRENE, SCLERODERMA (Disease), ETIOLOGY of diseases, RHEUMATOLOGY
Abstract

Objective. To evaluate in SSc, the frequency of digital lesions and the morphology, characteristics, natural course and time to healing of 1614 digital ulcers (DUs). [ABSTRACT FROM PUBLISHER]

Published
2010
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18. Sociodemographic factors in fibromyalgia: results from the Italian Fibromyalgia Registry

Author

Fabiola Atzeni, Alessandra Alciati, Laura Bazzichi, Marcello Govoni, Giovanni Biasi, Manuela Di Franco, Flavio Mozzani, Elisa Gremese, Lorenzo Dagna, Alberto Batticciotto, Fabio Fischetti, Roberto Giacomelli, Serena Guiducci, Giuliana Guggino, Mario Bentivegna, Roberto Gerli, Carlo Salvarani, Gianluigi Bajocchi, Marco Ghini, Florenzo Iannone, Valeria Giorgi, Marco Di Carlo, Sonia Farah, Sara Bonazza, Stefano Barbagli, Chiara Gioia, Noemi Giuliana Marino, Annunziata Capacci, Giulio Cavalli, Francesco Carubbi, Francesca Nacci, Ilenia Riccucci, Maurizio Cutolo, Luigi Sinigaglia, Piercarlo Sarzi-Puttini, Fausto Salaffi, Atzeni, Fabiola, Alciati, Alessandra, Bazzichi, Laura, Govoni, Marcello, Biasi, Giovanni, Di Franco, Manuela, Mozzani, Flavio, Gremese, Elisa, Dagna, Lorenzo, Batticciotto, Alberto, Fischetti, Fabio, Giacomelli, Roberto, Guiducci, Serena, Guggino, Giuliana, Bentivegna, Mario, Gerli, Roberto, Salvarani, Carlo, Bajocchi, Gianluigi, Ghini, Marco, Iannone, Florenzo, Giorgi, Valeria, Di Carlo, Marco, Farah, Sonia, Bonazza, Sara, Barbagli, Stefano, Gioia, Chiara, Marino, Noemi Giuliana, Capacci, Annunziata, Cavalli, Giulio, Carubbi, Francesco, Nacci, Francesca, Riccucci, Ilenia, Cutolo, Maurizio, Sinigaglia, Luigi, Sarzi-Puttini, Piercarlo, and Salaffi, Fausto

Subjects
Adult, Male, sociodemographic factors, Immunology, Reproducibility of Results, Severity of Illness Index, Rheumatology, Surveys and Questionnaires, Quality of Life, gender, Immunology and Allergy, Humans, Female, fibromyalgia, Registries, Chronic Pain, adult, female, humans, male, quality of life, registries, reproducibility of results, severity of illness index, surveys and questionnaires, chronic pain
Abstract

ObjectiveFibromyalgia (FM) is a chronic musculoskeletal pain syndrome of unknown aetiopathogenesis. Its development and maintenance are related to the interplay of biological, psychological, and contextual factors. Among the contextual factors, sociodemographic aspects are poorly elucidated. This study aimed to evaluate the relationships between sociodemographic/ clinical factors and symptom severity measures using a web-based registry of patients with FM.MethodsAdult patients with an ACR 2010/2011 diagnosis of FM underwent a clinical evaluation and were asked to complete questionnaires covering their sociodemographic data (gender, age, marital status, educational level), and disease-specific measures (the revised Fibromyalgia Impact Questionnaire (FIQR), and the Polysymptomatic Distress Scale (PDS)).ResultsData relating to 3,221 patients (3001 women and 220 men) was collected. The ANOVA showed significant difference in mean FIQR scores when the five marital conditions (cohabiter, married, separated/divorced, single, widowed) were compared ( F 3.321, p< 0.01). While males and females were found to have comparable FIQR scores, the interaction between gender and marital status indicated that separated/divorced males have higher FIQR scores (F 5.684, p=0.001). The multiple regression analysis demonstrated that patients who reported lower educational level experienced more severe FM symptoms, as scored with FIQR (p

Published
2022

19. The measurement of fibromyalgia severity: converting scores between the FIQR, the PSD and the FASmod

Author

Fausto, Salaffi, Marco, Di Carlo, Sonia, Farah, Manuela, Di Franco, Laura, Bazzichi, Gerolamo, Bianchi, Rosella, Tirri, Fabiola, Atzeni, Serena, Guiducci, Giuliana, Guggino, Roberto, Gorla, Fabio, Fischetti, Flavio, Mozzani, Giovanni, Biasi, Elisa, Gremese, Lorenzo, Dagna, Marcello, Govoni, Roberto, Giacomelli, Roberto, Gerli, Florenzo, Iannone, Maurizio, Cutolo, Frederick, Wolfe, Piercarlo, Sarzi-Puttini, Valeria, Giorgi, Salaffi, Fausto, Di Carlo, Marco, Farah, Sonia, Di Franco, Manuela, Bazzichi, Laura, Bianchi, Gerolamo, Tirri, Rosella, Atzeni, Fabiola, Guiducci, Serena, Guggino, Giuliana, Gorla, Roberto, Fischetti, Fabio, Mozzani, Flavio, Biasi, Giovanni, Gremese, Elisa, Dagna, Lorenzo, Govoni, Marcello, Giacomelli, Roberto, Gerli, Roberto, Iannone, Florenzo, Cutolo, Maurizio, Wolfe, Frederick, and Sarzi-Puttini, Piercarlo

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

The revised Fibromyalgia Impact Questionnaire (FIQR) is a widely used fibromyalgia severity assessment tool that was introduced in 2009 prior to the publication of the American College of Rheumatology (ACR) preliminary fibromyalgia criteria in 2010 and its revision in 2016. In 2020, the modified Fibromyalgia Assessment Scale (FASmod) was published. The Polysymptomatic Distress scale (PSD) of the fibromyalgia criteria and FASmod include assessments of pain location severity and can be used for diagnosis as well as in non-fibromyalgia patients. The aim of this study is to provide equations for the conversion of the FIQR scores to PSD and FASmod as an aid to understanding and sharing fibromyalgia severity information.3089 patients with fibromyalgia, diagnosed according to the ACR 2010/2011 criteria and belonging to the Italian Fibromyalgia Registry completed FIQR, FASmod and PSD questionnaires. Pearson's correlation coefficient was used to test the correlations between indices. The least square regression approach was used to produce predictive equations for each scale based on the remaining scales.FIQR was correlated with PSD (r=0.714) and FASmod (r=0.801); PSD and FASmod showed the highest correlation (r=0.897), expected since they assess the same constructs. Predictive equations showing a linear model were effective in producing mean cohort values, but individual predictions deviated substantially, precluding prediction in the individual patient.Conversion equations that allow for interconversion of multiple scales fibromyalgia severity assessment scales are produced. These can be useful in obtaining mean values for cohorts but are not accurate enough for use in individual patients.

Published
2022

20. Fibromyalgia severity according to age categories: results of a cross-sectional study from a large national database

Author

Marco Di Carlo, Sonia Farah, Laura Bazzichi, Fabiola Atzeni, Marcello Govoni, Giovanni Biasi, Manuela Di Franco, Flavio Mozzani, Elisa Gremese, Lorenzo Dagna, Alberto Batticciotto, Fabio Fischetti, Roberto Giacomelli, Serena Guiducci, Giuliana Guggino, Mario Bentivegna, Roberto Gerli, Carlo Salvarani, Gianluigi Bajocchi, Marco Ghini, Florenzo Iannone, Valeria Giorgi, Mariateresa Cirillo, Sara Bonazza, Stefano Barbagli, Chiara Gioia, Noemi Giuliana Marino, Annunziata Capacci, Giulio Cavalli, Antonella Cappelli, Francesco Carubbi, Francesca Nacci, Ilenia Riccucci, Maurizio Cutolo, Luigi Sinigaglia, Piercarlo Sarzi-Puttini, Fausto Salaffi, Di Carlo, Marco, Farah, Sonia, Bazzichi, Laura, Atzeni, Fabiola, Govoni, Marcello, Biasi, Giovanni, Di Franco, Manuela, Mozzani, Flavio, Gremese, Elisa, Dagna, Lorenzo, Batticciotto, Alberto, Fischetti, Fabio, Giacomelli, Roberto, Guiducci, Serena, Guggino, Giuliana, Bentivegna, Mario, Gerli, Roberto, Salvarani, Carlo, Bajocchi, Gianluigi, Ghini, Marco, Iannone, Florenzo, Giorgi, Valeria, Cirillo, Mariateresa, Bonazza, Sara, Barbagli, Stefano, Gioia, Chiara, Marino, Noemi Giuliana, Capacci, Annunziata, Cavalli, Giulio, Cappelli, Antonella, Carubbi, Francesco, Nacci, Francesca, Riccucci, Ilenia, Cutolo, Maurizio, Sinigaglia, Luigi, Sarzi-Puttini, Piercarlo, and Salaffi, Fausto

Subjects
Adult, Male, Settore MED/16 - REUMATOLOGIA, Adolescent, Immunology, Reproducibility of Results, Middle Aged, FAS 2019mod, Severity of Illness Index, Young Adult, Cross-Sectional Studies, Rheumatology, age, national database, Surveys and Questionnaires, Quality of Life, Immunology and Allergy, Humans, disease severity, Female, fibromyalgia, FIQR
Abstract

ObjectiveThe role of age in influencing the severity of fibromyalgia (FM) is still controversial. The aim of this study is to define the contribution of age in the severity of FM from data from a large national database.MethodsThis cross-sectional study included adult patients with FM diagnosed according to the 2010/2011 American College of Rheumatology criteria. Disease severity was assessed with the revised Fibromyalgia Impact Questionnaire (FIQR) and the modified Fibromyalgia Assessment Status (FAS 2019mod). Patients were grouped into five age categories (between 18-40 years, between 41- 50 years, between 51-60 years, between 61-70 years, and =71 years). Differences in disease severity between groups were assessed by one-way analysis of variance (ANOVA).ResultsThe study included 2889 patients (199 males and 2690 females), mean age of 52.58 (+/- 11.82) years, with a mean FIQR score of 59.22 (+/- 22.98) and a mean FAS 2019mod of 25.50 (+/- 8.66). Comparing the mean values of the various indices between age categories, there were no statistically significant differences between the groups for FIQR total score and FAS 2019mod. However, the 60-70 years category showed the lowest scores for both scales. The main difference emerged for the FIQR physical function subscale, where the =71 years category showed significantly higher scores (p

Published
2022

21. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

Author

Rossella, De Angelis, Dilia, Giuggioli, Gianluigi, Bajocchi, Lorenzo, Dagna, Giovanni, Zanframundo, Rosario, Foti, Fabio, Cacciapaglia, Giovanna, Cuomo, Alarico, Ariani, Edoardo, Rosato, Serena, Guiducci, Francesco, Girelli, Valeria, Riccieri, Elisabetta, Zanatta, Silvia, Bosello, Ilaria, Cavazzana, Francesca, Ingegnoli, Maria De, Santis, Giuseppe, Murdaca, Giuseppina, Abignano, Nicoletta, Romeo, Alessandra, Della Rossa, Maurizio, Caminiti, Annamaria, Iuliano, Giovanni, Ciano, Lorenzo, Beretta, Gianluca, Bagnato, Ennio, Lubrano, Ilenia, De Andres, Alessandro, Giollo, Marta, Saracco, Cecilia, Agnes, Federica, Lumetti, Amelia, Spinella, Luca, Magnani, Corrado, Campochiaro, Giacomo, De Luca, Veronica, Codullo, Elisa, Visalli, Francesco, Masini, Antonietta, Gigante, Silvia, Bellando-Randone, Greta, Pellegrino, Erika, Pigatto, Francesca, Dall'Ara, Maria Grazia, Lazzaroni, Elena, Generali, Gianna, Mennillo, Simone, Barsotti, Giuseppa Pagano, Mariano, Francesca, Calabrese, Federica, Furini, Licia, Vultaggio, Simone, Parisi, Clara Lisa, Peroni, Anna Maria, Risa, Davide, Rozza, Anna, Zanetti, Greta, Carrara, Giampiero, Landolfi, Carlo Alberto, Scirè, Gerolamo, Bianchi, Enrico, Fusaro, Gian Domenico, Sebastiani, Marcello, Govoni, Salvatore, D'Angelo, Franco, Cozzi, Andrea, Doria, Florenzo, Iannone, Carlo, Salvarani, Marco, Matucci-Cerinic, Clodoveo, Ferri, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Dall'Ara, F, Lazzaroni, M, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Risa, A, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri, C, de Angelis, R., Giuggioli, D., Bajocchi, G., Dagna, L., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Guiducci, S., Girelli, F., Riccieri, V., Zanatta, E., Bosello, S., Cavazzana, I., Ingegnoli, F., de Santis, M., Murdaca, G., Abignano, G., Romeo, N., Rossa, A. D., Caminiti, M., Iuliano, A., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., de Andres, I., Giollo, A., Saracco, M., Agnes, C., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., de Luca, Giacomo., Codullo, V., Visalli, E., Masini, F., Gigante, A., Bellando-Randone, S., Pellegrino, G., Pigatto, E., Dall'Ara, F., Lazzaroni, M. G., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Calabrese, F., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Risa, A. M., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, G. D., Govoni, M., D'Angelo, S., Cozzi, F., Doria, A., Iannone, F., Salvarani, C., Matucci-Cerinic, M., Ferri, C., De Angelis, Rossella, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Guiducci, Serena, Girelli, Francesco, Riccieri, Valeria, Zanatta, Elisabetta, Bosello, Silvia, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Masini, Francesco, Gigante, Antonietta, Bellando-Randone, Silvia, Pellegrino, Greta, Pigatto, Erika, Dall'Ara, Francesca, Lazzaroni, Maria Grazia, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Pagano Mariano, Giuseppa, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Risa, Anna Maria, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Giampiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Doria, Andrea, Iannone, Florenzo, Salvarani, Carlo, Matucci-Cerinic, Marco, and Ferri, Clodoveo

Subjects
Male, medicine.medical_specialty, Vital capacity, Settore MED/16 - REUMATOLOGIA, Cross-sectional study, Immunology, Left, Socio-culturale, scleroderma, sex, systemic sclerosis, Disease, Ventricular Function, Left, Scleroderma, Systemic sclerosi, Rheumatology, Internal medicine, Sicca syndrome, Sex, Systemic sclerosis, Cross-Sectional Studies, Female, Humans, Italy, Registries, Sex Characteristics, Stroke Volume, Scleroderma, Systemic, Sjogren's Syndrome, medicine, LS8_2, Immunology and Allergy, Ventricular Function, Honeycombing, skin and connective tissue diseases, Ejection fraction, integumentary system, business.industry, Systemic, medicine.disease, Cohort, business
Abstract

ObjectiveThere is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics.MethodsA multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared.ResultsThe overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs.ConclusionOur study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.

Published
2022

22. Spectrum of short-term inflammatory musculoskeletal manifestations after COVID-19 vaccine administration: a report of 66 cases

Author

Francesca Motta, Veronica Brusi, Corrado Campochiaro, Lorenzo Dagna, Enrico Fusaro, Ilenia Di Cola, Clodoveo Ferri, Fabio Cacciapaglia, Caterina Bruno, Rosario Foti, Riccardo Meliconi, Francesca Francioso, Felice Galluccio, Serena Guiducci, Vincenzo Raimondo, Jacopo Ciaffi, Annamaria Iagnocco, Giovanna Cuomo, Giacomo De Luca, Antonio Tavoni, Francesco Ursini, Roberto Giacomelli, Maria De Santis, Silvia Bilia, Piero Ruscitti, Salvatore D'Angelo, Florenzo Iannone, Domenico Olivo, Martina D'Onghia, Rossella De Angelis, Marcella Visentini, Luana Mancarella, E. Pigatto, Ursini, F., Ruscitti, P., Raimondo, V., De Angelis, R., Cacciapaglia, F., Pigatto, E., Olivo, D., Di Cola, I., Galluccio, F., Francioso, F., Foti, R., Tavoni, A., D'Angelo, S., Campochiaro, C., Motta, F., De Santis, M., Bilia, S., Bruno, C., DE LUCA, Giacomo, Visentini, M., Ciaffi, J., Mancarella, L., Brusi, V., D'Onghia, M., Cuomo, G., Fusaro, E., Dagna, L., Guiducci, S., Meliconi, R., Iannone, F., Iagnocco, A., Giacomelli, R., Ferri, C., Ursini, Francesco, Ruscitti, Piero, Raimondo, Vincenzo, De Angelis, Rossella, Cacciapaglia, Fabio, Pigatto, Erika, Olivo, Domenico, Di Cola, Ilenia, Galluccio, Felice, Francioso, Francesca, Foti, Rosario, Tavoni, Antonio, D'Angelo, Salvatore, Campochiaro, Corrado, Motta, Francesca, De Santis, Maria, Bilia, Silvia, Bruno, Caterina, De Luca, Giacomo, Visentini, Marcella, Ciaffi, Jacopo, Mancarella, Luana, Brusi, Veronica, D'Onghia, Martina, Cuomo, Giovanna, Fusaro, Enrico, Dagna, Lorenzo, Guiducci, Serena, Meliconi, Riccardo, Iannone, Florenzo, Iagnocco, Annamaria, Giacomelli, Roberto, Ferri, Clodoveo, Ursini, F, Ruscitti, P, Raimondo, V, De Angelis, R, Cacciapaglia, F, Pigatto, E, Olivo, D, Di Cola, I, Galluccio, F, Francioso, F, Foti, R, Tavoni, A, D'Angelo, S, Campochiaro, C, Motta, F, De Santis, M, Bilia, S, Bruno, C, De Luca, G, Visentini, M, Ciaffi, J, Mancarella, L, Brusi, V, D'Onghia, M, Cuomo, G, Fusaro, E, Dagna, L, Guiducci, S, Meliconi, R, Iannone, F, Iagnocco, A, Giacomelli, R, and Ferri, C

Subjects
Male, medicine.medical_specialty, COVID-19 Vaccines, Coronavirus disease 2019 (COVID-19), Immunology, Population, Arthritis, medicine.disease_cause, General Biochemistry, Genetics and Molecular Biology, polymyalgia rheumatica, Immune system, Rheumatology, Rheumatic Diseases, Pandemic, medicine, Immunology and Allergy, Humans, Musculoskeletal Diseases, Covid-19, arthritis, vaccination, Intensive care medicine, Adverse effect, education, education.field_of_study, business.industry, SARS-CoV-2, COVID-19, Female, Middle Aged, medicine.disease, Vaccination, arthriti, Molecular mimicry, business
Abstract

In the past months, mass vaccination represented the turning point of the global battle against the COVID-19 pandemic, an unprecedented challenge for physicians, healthcare professionals, health systems and pharmaceutical companies. More than 6 billion doses of vaccine have been administered to date, covering nearly 50% of the world’s population. Although the vaccination campaign is still thwarted by spread of fake news disseminated by a ubiquitous antivaxxer movement, accumulating real-life data1 confirm the favourable safety profile already demonstrated in phase III clinical trials.2 Despite the lack of a steady literature evidence,3 the potential role of vaccines in promoting autoimmunity continues to intrigue many researchers. The theoretical basis of this association relies on the possible molecular mimicry between macromolecular components of the vaccine and specific human proteins and the exuberant immune response elicited by adjuvants contained in vaccines.4 Adverse events (AEs) associated with COVID-19 vaccines are usually mild and mainly restricted to injection site reactions. Interestingly, among systemic AEs, arthralgia is one of the most common.2 To the best of our knowledge, only isolated cases5 of arthritis developed after COVID-19 …

Published
2022

23. The role of chest CT in deciphering interstitial lung involvement: systemic sclerosis versus COVID-19

Author

Jelena Blagojevic, Francesca Wanda Rossi, Alessandro Bartoloni, Cosimo Nardi, S. Tomassetti, Martina Orlandi, Alberto Moggi-Pignone, Yannick Allanore, L. Dagna, Stefano Palmucci, Carlo Vancheri, Marco Matucci-Cerinic, Francesca Della Casa, Marco Confalonieri, Federico Lavorini, Amato de Paulis, Lorenzo Tofani, Gloria Taliani, Virginia Vegni, Dinesh Khanna, Vittorio Miele, Alberto Pesci, Barbara Ruaro, C. Campochiaro, Lorenzo Zammarchi, Giovanni Morana, Michele Spinicci, Gianluca Sambataro, Antonella Caminati, Silvia Bellando-Randone, Daniela Melchiorre, Cosimo Bruni, Nicholas Landini, Francesco De Cobelli, Masataka Kuwana, Giacomo De Luca, Sergio Harari, Stefano Colagrande, Fabio Melchiorre, Edoardo Cavigli, Serena Guiducci, Christopher P. Denton, Fabrizio Luppi, Michael Hughes, Marco Albanesi, Orlandi, Martina, Landini, Nichola, Sambataro, Gianluca, Nardi, Cosimo, Tofani, Lorenzo, Bruni, Cosimo, Bellando-Randone, Silvia, Blagojevic, Jelena, Melchiorre, Daniela, Hughes, Michael, Denton, Christopher P, Luppi, Fabrizio, Ruaro, Barbara, Della Casa, Francesca, Rossi, Francesca W, De Luca, Giacomo, Campochiaro, Corrado, Spinicci, Michele, Zammarchi, Lorenzo, Tomassetti, Sara, Caminati, Antonella, Cavigli, Edoardo, Albanesi, Marco, Melchiorre, Fabio, Palmucci, Stefano, Vegni, Virginia, Guiducci, Serena, Moggi-Pignone, Alberto, Allanore, Yannick, Bartoloni, Alessandro, Confalonieri, Marco, Dagna, Lorenzo, De Cobelli, Francesco, De Paulis, Amato, Harari, Sergio, Khanna, Dinesh, Kuwana, Masataka, Taliani, Gloria, Lavorini, Federico, Miele, Vittorio, Morana, Giovanni, Pesci, Alberto, Vancheri, Carlo, Colagrande, Stefano, Matucci-Cerinic, Marco, Denton, Christopher P., Rossi, Francesca W., Decobelli, Francesco, Depaulis, Amato, Orlandi, M, Landini, N, Sambataro, G, Nardi, C, Tofani, L, Bruni, C, Bellando-Randone, S, Blagojevic, J, Melchiorre, D, Hughes, M, Denton, C, Luppi, F, Ruaro, B, Della Casa, F, Rossi, F, De Luca, G, Campochiaro, C, Spinicci, M, Zammarchi, L, Tomassetti, S, Caminati, A, Cavigli, E, Albanesi, M, Melchiorre, F, Palmucci, S, Vegni, V, Guiducci, S, Moggi-Pignone, A, Allanore, Y, Bartoloni, A, Confalonieri, M, Dagna, L, De Cobelli, F, De Paulis, A, Harari, S, Khanna, D, Kuwana, M, Taliani, G, Lavorini, F, Miele, V, Morana, G, Pesci, A, Vancheri, C, Colagrande, S, Matucci-Cerinic, M, Orlandi, M., Landini, N., Sambataro, G., Nardi, C., Tofani, L., Bruni, C., Bellando-Randone, S., Blagojevic, J., Melchiorre, D., Hughes, M., Denton, C. P., Luppi, F., Ruaro, B., Della Casa, F., Rossi, F. W., De Luca, G., Campochiaro, C., Spinicci, M., Zammarchi, L., Tomassetti, S., Caminati, A., Cavigli, E., Albanesi, M., Melchiorre, F., Palmucci, S., Vegni, V., Guiducci, S., Moggi-Pignone, A., Allanore, Y., Bartoloni, A., Confalonieri, M., Dagna, L., Decobelli, F., de Paulis, A., Harari, S., Khanna, D., Kuwana, M., Taliani, G., Lavorini, F., Miele, V., Morana, G., Pesci, A., Vancheri, C., Colagrande, S., and Matucci-Cerinic, M.

Subjects
medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Fibrosi, systemic sclerosis, education, Chest ct, Stock options, COVID-19, COVID-19 pneumonia, interstitial lung disease, lung CT scan, Computed tomography, Institutional ethics, COVID-19 Testing, Rheumatology, Fibrosis, Medicine, Humans, Pharmacology (medical), Lung, health care economics and organizations, Scleroderma, Systemic, Competing interests, medicine.diagnostic_test, business.industry, Interstitial lung disease, medicine.disease, Lung involvement, Peripheral, Clinical Practice, Pneumonia, Family medicine, Radiology, Differential diagnosis, business, Lung Diseases, Interstitial, Tomography, X-Ray Computed, systemic sclerosi, Human
Abstract

Background: In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 pneumonia from a progression of interstitial lung disease (ILD) secondary to Systemic sclerosis (SSc). The aim of the present study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia. Methods: This multicentric study included 22 international readers divided in the radiologist group (RAD) and non-radiologist group (nRAD). A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study. Findings: Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT features most frequently associated with SSc-ILD. The CT features most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p

Published
2022

24. The association between body mass index and fibromyalgia severity: data from a cross-sectional survey of 2339 patients

Author

Maurizio Cutolo, Piercarlo Sarzi-Puttini, Marcello Govoni, Francesca Nacci, Ilenia Riccucci, Fabio Fischetti, Sonia Farah, Mario Bentivegna, Giulio Cavalli, Elisa Gremese, A. Capacci, Sara Bonazza, Antonella Cappelli, Valeria Giorgi, Carlo Salvarani, Chiara Gioia, Stefano Barbagli, Roberto Giacomelli, Gianluigi Bajocchi, Marco Ghini, Lorenzo Dagna, Manuela Di Franco, Luigi Sinigaglia, Francesco Carubbi, Florenzo Iannone, Roberto Gerli, F. Mozzani, Marco Di Carlo, Giovanni Biasi, Noemi Giuliana Marino, Fausto Salaffi, Fabiola Atzeni, Giuliana Guggino, Alberto Batticciotto, Alessandra Alciati, Serena Guiducci, Laura Bazzichi, Atzeni F., Alciati A., Salaffi F., Di Carlo M., Bazzichi L., Govoni M., Biasi G., Di Franco M., Mozzani F., Gremese E., Dagna L., Batticciotto A., Fischetti F., Giacomelli R., Guiducci S., Guggino G., Bentivegna M., Gerli R., Salvarani C., Bajocchi G., Ghini M., Iannone F., Giorgi V., Farah S., Bonazza S., Barbagli S., Gioia C., Marino N.G., Capacci A., Cavalli G., Cappelli A., Carubbi F., Nacci F., Riccucci I., Cutolo M., Sinigaglia L., Sarzi-Puttini P., Atzeni, Fabiola, Alciati, Alessandra, Salaffi, Fausto, Di Carlo, Marco, Bazzichi, Laura, Govoni, Marcello, Biasi, Giovanni, Di Franco, Manuela, Mozzani, Flavio, Gremese, Elisa, Dagna, Lorenzo, Batticciotto, Alberto, Fischetti, Fabio, Giacomelli, Roberto, Guiducci, Serena, Guggino, Giuliana, Bentivegna, Mario, Gerli, Roberto, Salvarani, Carlo, Bajocchi, Gianluigi, Ghini, Marco, Iannone, Florenzo, Giorgi, Valeria, Farah, Sonia, Bonazza, Sara, Barbagli, Stefano, Gioia, Chiara, Marino, Noemi Giuliana, Capacci, Annunziata, Cavalli, Giulio, Cappelli, Antonella, Carubbi, Francesco, Nacci, Francesca, Riccucci, Ilenia, Cutolo, Maurizio, Sinigaglia, Luigi, and Sarzi-Puttini, Piercarlo

Subjects
medicine.medical_specialty, obesity, Cross-sectional study, Revised Fibromyalgia Impact Questionnaire, Overweight, NO, 03 medical and health sciences, BMI, 0302 clinical medicine, Rheumatology, Internal medicine, Fibromyalgia, medicine, LS4_5, 030212 general & internal medicine, 030203 arthritis & rheumatology, business.industry, medicine.disease, Obesity, Distress, clinical severity, widespread pain, Original Article, fibromyalgia, Underweight, medicine.symptom, AcademicSubjects/MED00010, business, Body mass index
Abstract

Objective Various studies have shown that overweight and obesity are central features of FM, but the real impact of a high BMI on clinical severity in patients with FM is still controversial. The aim of this study was to analyse the relationships between BMI categories and measures of symptom severity and functional impairment using data from a Web-based registry of patients with FM. Methods Adult patients with an ACR 2010/2011 diagnosis of FM underwent a complete physical examination and laboratory tests and were asked to complete a package of questionnaires covering their sociodemographic and treatment details, in addition to the following disease-specific questionnaires: the revised Fibromyalgia Impact Questionnaire (FIQR), the modified Fibromyalgia Assessment Status questionnaire (ModFAS) and the Polysymptomatic Distress Scale (PDS). Results A total of 2339 patients were recruited and divided into two weight categories, underweight/normal (U/N, n = 1127, 48.2%) and overweight/obese (O/O, n = 1212, 51.8%). The total and subscales of FIQR, ModFAS and PSD scores were significantly higher in the O/O patients, as were all the mean scores of the individual FIQR items (P Conclusion Our findings demonstrate that O/O patients with FM are significantly more impaired than U/N patients in all the symptomatological and functional domains as measured using the FIQR, ModFAS and PDS, thus suggesting that being O/O has an additional effect on symptoms and function.

Published
2021

25. Definition of fibromyalgia severity: findings from a cross-sectional survey of 2339 Italian patients

Author

Maurizio Masullo, A. Capacci, Roberto Giacomelli, Fabio Fischetti, Mariateresa Cirillo, Giuliana Guggino, Giovanni Biasi, Lorenzo Dagna, Manuela Di Franco, Marco Ghini, Maurizio Cutolo, Daniele Santilli, Sara Bonazza, F. Mozzani, Carlo Salvarani, Elisa Gremese, Ilenia Riccucci, Gianluigi Bajocchi, Alberto Batticciotto, Laura Bazzichi, Giulio Cavalli, Marco Di Carlo, Florenzo Iannone, Stefano Barbagli, Sonia Farah, Serena Guiducci, Roberto Gerli, Marcello Govoni, Francesca Nacci, Francesco Carubbi, Piercarlo Sarzi-Puttini, Mario Bentivegna, Bianca Maria Polizzi, Luigi Sinigaglia, Fabiola Atzeni, Valeria Giorgi, Chiara Gioia, Fausto Salaffi, Salaffi, Fausto, Di Carlo, Marco, Bazzichi, Laura, Atzeni, Fabiola, Govoni, Marcello, Biasi, Giovanni, Di Franco, Manuela, Mozzani, Flavio, Gremese, Elisa, Dagna, Lorenzo, Batticciotto, Alberto, Fischetti, Fabio, Giacomelli, Roberto, Guiducci, Serena, Guggino, Giuliana, Bentivegna, Mario, Gerli, Roberto, Salvarani, Carlo, Bajocchi, Gianluigi, Ghini, Marco, Iannone, Florenzo, Giorgi, Valeria, Farah, Sonia, Cirillo, Mariateresa, Bonazza, Sara, Barbagli, Stefano, Gioia, Chiara, Santilli, Daniele, Capacci, Annunziata, Cavalli, Giulio, Carubbi, Francesco, Nacci, Francesca, Riccucci, Ilenia, Sinigaglia, Luigi, Masullo, Maurizio, Polizzi, Bianca Maria, Cutolo, Maurizio, Sarzi-Puttini, Piercarlo, Salaffi F., Di Carlo M., Bazzichi L., Atzeni F., Govoni M., Biasi G., Di Franco M., Mozzani F., Gremese E., Dagna L., Batticciotto A., Fischetti F., Giacomelli R., Guiducci S., Guggino G., Bentivegna M., Gerli R., Salvarani C., Bajocchi G., Ghini M., Iannone F., Giorgi V., Farah S., Cirillo M., Bonazza S., Barbagli S., Gioia C., Santilli D., Capacci A., Cavalli G., Carubbi F., Nacci F., Riccucci I., Sinigaglia L., Masullo M., Polizzi B.M., Cutolo M., Sarzi-Puttini P., and Maria Polizzi, Bianca

Subjects
Male, Percentile, Settore MED/16 - REUMATOLOGIA, Fibromyalgia, Cross-sectional study, severity, Disease, Severity of Illness Index, Economica, 0302 clinical medicine, modified, Interquartile range, Retrospective Studie, Surveys and Questionnaires, Surveys and Questionnaire, Pharmacology (medical), 030212 general & internal medicine, cut-off points, Pain Measurement, Fibromyalgia Assessment Status, fibromyalgia, polysymptomatic distress scale, revised Fibromyalgia Impact Questionnaire, Cross-Sectional Studies, Female, Follow-Up Studies, Humans, Incidence, Italy, Middle Aged, Prognosis, Retrospective Studies, Quality of Life, Distress, Human, medicine.medical_specialty, Prognosi, Revised Fibromyalgia Impact Questionnaire, Follow-Up Studie, 03 medical and health sciences, Rheumatology, Internal medicine, Severity of illness, cut-off point, medicine, 030203 arthritis & rheumatology, Cross-Sectional Studie, business.industry, medicine.disease, Fibromyalgia Assessment Statu, fibromyalgia assessment status, revised fibromyalgia impact questionnaire, business
Abstract

Objective To establish optimal cut-off values for the scores of the revised Fibromyalgia Impact Questionnaire (FIQR), the modified Fibromialgia Assessment Scale (FAS 2019mod), and the Polysymptomatic Distress Scale (PDS) in order to distinguish five levels of FM disease severity. Methods Consecutive FM patients were evaluated with the three clinimetric indices, and each patient was required to answer the anchor question: ‘In general, would you say your health is 1 = very good, 2 = good, 3 = fair, 4 = poor, or 5 = very poor?’—which represented the external criterion. Cut-off points were established through the interquartile reconciliation approach. Results The study sample consisted of 2181 women (93.2%) and 158 men (6.8%), with a mean age of 51.9 (11.5) years, and mean disease duration was 7.3 (6.9) years. The overall median FIQR, FAS 2019 mod and PDS scores (25th–75th percentiles) were respectively 61.16 (41.16–77.00), 27.00 (19.00–32.00) and 19.0 (13.00–24.00). Reconciliation of the mean 75th and 25th percentiles of adjacent categories defined the severity states for FIQR: 0–23 for remission, 24–40 for mild disease, 41–63 for moderate disease, 64–82 for severe disease and >83 for very severe disease; FAS 2019 mod: 0–12 for remission, 13–20 for mild disease, 21–28 for moderate disease, 29–33 for severe disease and >33 for very severe disease; PDS: 0–5 for remission, 6–15 for mild disease, 16–20 for moderate disease, 21–25 for severe disease and >25 for very severe disease. Conclusions Disease severity cut-offs can represent an important improvement in interpreting FM.

Published
2021

26. Elevated serum levels of sonic hedgehog are associated with fibrotic and vascular manifestations in systemic sclerosis

Author

Serena Vettori, Andreas Ramming, Gabriele Valentini, Jérôme Avouac, Ulf Müller-Ladner, Britta Maurer, Jörg H W Distler, Serena Guiducci, Marco Matucci-Cerinic, Dörte Huscher, Yannick Allanore, Oliver Distler, Veronika K. Jaeger, Florian M P Meier, Georg Schett, Ulrich A Walker, Gabriela Riemekasten, Elise Siegert, Christian Beyer, Christina Bergmann, Beyer, Christian, Huscher, Dörte, Ramming, Andrea, Bergmann, Christina, Avouac, Jerome, Guiducci, Serena, Meier, Florian, Vettori, Serena, Siegert, Elise, Jaeger, Veronika K, Maurer, Britta, Riemekasten, Gabriela, Walker, Ulrich, Müller Ladner, Ulf, Valentini, Gabriele, Matucci Cerinic, Marco, Allanore, Yannick, Distler, Oliver, Schett, Georg, Distler, Jörg H. W., University of Zurich, and Distler, Jörg H W

Subjects
0301 basic medicine, medicine.medical_specialty, Pathology, animal structures, 2745 Rheumatology, Immunology, 610 Medicine & health, Genetics and Molecular Biology, Systemic Sclerosis, Autoimmune Disease, General Biochemistry, Genetics and Molecular Biology, Scleroderma, Pathogenesis, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Fibrosis, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, Immunology and Allergy, Sonic hedgehog, skin and connective tissue diseases, 030203 arthritis & rheumatology, 2403 Immunology, integumentary system, biology, business.industry, Case-control study, 10051 Rheumatology Clinic and Institute of Physical Medicine, medicine.disease, Phenotype, Pulmonary hypertension, 030104 developmental biology, embryonic structures, General Biochemistry, biology.protein, 2723 Immunology and Allergy, Fibroblast, business
Abstract

Fibrotic tissue remodelling and vascular alterations cause high morbidity and mortality in patients with systemic sclerosis (SSc).1 2 While the pathogenesis of SSc is only partially understood, recent evidence demonstrates a fundamental role of hedgehog signalling in fibrotic disease manifestations. Hedgehog signalling is activated in fibrotic SSc skin, and it induces an SSc-like phenotype in resting fibroblasts.3 Overexpression of sonic hedgehog (SHH) induces skin fibrosis, whereas inhibition of hedgehog signalling ameliorates experimental fibrosis.4 Based on these experimental findings, this European Scleroderma Trials and Research group (EUSTAR) multicentre cooperative study investigates whether circulating SHH may serve as a surrogate marker for a more severe disease phenotype in patients with SSc. We decided to evaluate SHH serum levels rather than total circulating SHH. Measurements of SHH serum levels are easy and robust but may not assess particulated SHH in various different extracellular vesicles. …

Published
2018

27. The 'myth' of loss of angiogenesis in systemic sclerosis: a pivotal early pathogenetic process or just a late unavoidable event?

Author

Inês Chora, Cosimo Bruni, Serena Guiducci, Marco Matucci-Cerinic, Gemma Lepri, Silvia Bellando-Randone, Mirko Manetti, Amato de Paulis, Matucci Cerinic, Marco, Manetti, Mirko, Bruni, Cosimo, Chora, Ine, Bellando Randone, Silvia, Lepri, Gemma, DE PAULIS, Amato, and Guiducci, Serena

Subjects
0301 basic medicine, Pathology, medicine.medical_specialty, lcsh:Diseases of the musculoskeletal system, Angiogenesis, Late loss, Scleroderma, Systemic sclerosis, Immunology and Allergy, Rheumatology, Immunology, Pathogenesis, 03 medical and health sciences, 0302 clinical medicine, Humans, Medicine, Process (anatomy), 030203 arthritis & rheumatology, Scleroderma, Systemic, Neovascularization, Pathologic, business.industry, Regeneration (biology), medicine.disease, Endothelial stem cell, Angiogenesi, 030104 developmental biology, Commentary, lcsh:RC925-935, business
Abstract

Systemic sclerosis is considered a disease dominated by a “loss of angiogenesis”, although in its early phases evidence indicates a disturbed angiogenic response only. In fact, microvascular changes are primarily due to endothelial cell injury, triggering downstream significant enlargement of the capillary in an inflammatory environment, followed by capillary rupture (microhemorrhages). Subsequent pro-angiogenic efforts lead to an aberrant angiogenesis and, eventually, to a total loss of vessel repair and regeneration (loss of angiogenesis). This clearly suggests that the pathogenetic process has a steady progression: from an early excessive pro-angiogenesis, to an aberrant microvascular regeneration, then ending with a late loss of angiogenesis. Herein, we suggest the loss of angiogenesis should not be considered as an overall “myth” characterizing systemic sclerosis but as a very late event of the vascular pathogenesis. Future research should be oriented essentially on the earlier phases dominated by excessive pro-angiogenesis and microvascular aberration.

Published
2017

28. Evidence for a Derangement of the Microvascular System in Patients with a Very Early Diagnosis of Systemic Sclerosis

Author

Raquel Costa, Raquel Soares, Amato de Paulis, Serena Guiducci, Gemma Lepri, Inês Chora, Marco Matucci-Cerinic, Mirko Manetti, C. Mazzotta, Alice Cortez, Eloisa Romano, V. A. Machado, Cosimo Bruni, Chora, Inê, Romano, Eloisa, Manetti, Mirko, Mazzotta, Celestina, Costa, Raquel, Machado, Vera, Cortez, Alice, Bruni, Cosimo, Lepri, Gemma, Guiducci, Serena, De Paulis, Amato, Soares, Raquel, and Matucci-Cerinic, Marco

Subjects
0301 basic medicine, Adult, Male, Vascular Endothelial Growth Factor A, Pathology, medicine.medical_specialty, Angiogenesis, medicine.medical_treatment, Immunology, Neovascularization, Physiologic, ANGIOGENESIS, VERY EARLY DIAGNOSIS OF SYSTEMIC SCLEROSIS, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Cell Movement, medicine, Immunology and Allergy, Humans, Endothelial dysfunction, Aged, Cell Proliferation, Skin, 030203 arthritis & rheumatology, Tube formation, Aged, 80 and over, Matrigel, Wound Healing, Scleroderma, Systemic, business.industry, Cell growth, Growth factor, Endothelial Cells, Middle Aged, medicine.disease, Neuropilin-1, Blot, Endothelial stem cell, 030104 developmental biology, Early Diagnosis, SYSTEMIC SCLEROSIS, Microvessels, Female, business
Abstract

Objective.To investigate whether patients with a very early diagnosis of systemic sclerosis (VEDOSS) may already present circulating markers and in vitro signs of microvascular dysfunction.Methods.Serum samples were obtained from 55 patients with systemic sclerosis (SSc), 25 patients with VEDOSS, and 55 matched healthy controls (HC). Serum levels of pan-vascular endothelial growth factor (VEGF) and soluble neuropilin-1 (sNRP-1) were measured by ELISA. Human dermal microvascular endothelial cells (H-MVEC) were cultured and stimulated with SSc, VEDOSS, and HC sera. Protein expression of NRP-1 was analyzed by Western blotting, cell proliferation by 5′-bromodeoxyuridine assay, migration capacity by wound-healing assay, and capillary-like tube formation by Matrigel assay.Results.Serum levels of pan-VEGF were increased in patients with VEDOSS and SSc versus HC (p = 0.05 and p = 0.003, respectively). Serum levels of sNRP-1 were significantly reduced in patients with VEDOSS and SSc compared with controls (p = 0.012 and p = 0.027, respectively). NRP-1 expression was decreased in H-MVEC stimulated with VEDOSS sera (p < 0.001 vs HC). Proliferation was reduced in H-MVEC stimulated either with VEDOSS or SSc sera in comparison with HC sera (p = 0.015 and p = 0.043, respectively). Wound healing was compromised in H-MVEC stimulated with VEDOSS and SSc sera versus HC sera (p < 0.01 for both). Capillarogenesis was decreased in H-MVEC stimulated with VEDOSS sera (p < 0.01) and SSc sera (p < 0.001) compared with cells stimulated with HC sera.Conclusion.Similar to patients with SSc, patients with VEDOSS already present biological signs of endothelial dysfunction. Our data demonstrate that VEDOSS sera significantly modify endothelial cell behavior and impair the angiogenic potential of the microvascular system.

Published
2017

29. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group

Author

Ulf Müller-Ladner, Beat Fankhauser, Valeria Riccieri, Marco Matucci-Cerinic, Christopher P. Denton, Jörg Umbricht, Brigitte Krummel-Lorenz, Franco Cozzi, László Czirják, Jérôme Avouac, Ulrich A Walker, Alessandra Vacca, Armando Gabrielli, Serena Vettori, Yannick Allanore, Marc Frerix, Francesco Del Galdo, Giuseppina Abignano, Paloma García de la Peña Lefebvre, Dörte Huscher, Veronika Lóránd, Carina Mihai, Ingo H. Tarner, Britta Maurer, Gabriele Valentini, Elise Siegert, T. Schmeiser, Nicolas Hunzelmann, Simona Rednic, G. Riemekasten, Beata Garay Toth, Serena Guiducci, Svetlana I. Nihtyanova, Codrina Ancuta, Sule Yavuz, Oliver Distler, Vanessa Smith, Anastasia Zakharova, Veronika K. Jaeger, Duska Martinovic, Jaeger, Veronika K, Distler, Oliver, Maurer, Britta, Czirják, Laszlo, Lóránd, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Denton, Christopher, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jerome, Riemekasten, Gabriele, Siegert, Elise, Huscher, Dörte, Matucci Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H, Garay Toth, Beata, Fankhauser, Beat, Umbricht, Jörg, Zakharova, Anastasia, Mihai, Carina, Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicola, Rednic, Simona, Vacca, Alessandra, Schmeiser, Tim, Riccieri, Valeria, García de la Peña Lefebvre, Paloma, Gabrielli, Armando, Krummel Lorenz, Brigitte, Martinovic, Duska, Ancuta, Codrina, Smith, Vanessa, Müller Ladner, Ulf, and Walker, Ulrich A.

Subjects
medicine.medical_specialty, functional disability, systemic sclerosis, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Quality of life, Internal medicine, Severity of illness, medicine, Functional disability, Predictors of disability, Scleroderma health assessment questionnaire, Systemic sclerosis, Europe, Gastrointestinal Diseases, Humans, Hypertension, Pulmonary, Longitudinal Studies, Muscle Weakness, Pain Measurement, Prospective Studies, Regression Analysis, Risk Factors, Scleroderma, Systemic, Severity of Illness Index, Skin Ulcer, Activities of Daily Living, Disability Evaluation, Quality of Life, Sickness Impact Profile, Pharmacology (medical), 030212 general & internal medicine, Prospective cohort study, 030203 arthritis & rheumatology, business.industry, predictors of disability, scleroderma health assessment questionnaire, Muscle weakness, sistemska skleroza, funkcionalna invalidnost, zdravstveni upitnik, prediktori invaliditeta, Skin ulcer, medicine.disease, Pulmonary hypertension, Rheumatoid arthritis, Cohort, Physical therapy, Functional disability, systemic sclerosis, medicine.symptom, business
Abstract

Objectives: The multisystem manifestations of SSc can greatly impact patients' quality of life. The aim of this study was to identify factors associated with disability in SSc. Methods: SSc patients from the prospective DeSScipher cohort who had completed the scleroderma health assessment questionnaire (SHAQ), a disability score that combines the health assessment questionnaire and five visual analogue scales, were included in this analysis. The effect of factors possibly associated with disability was analysed with multiple linear regressions. Results: The mean SHAQ and HAQ scores of the 944 patients included were 0.87 ( s . d . = 0.66) and 0.92 ( s . d . = 0.78); 59% of the patients were in the mild to moderate difficulty SHAQ category (0 ⩽ SHAQ < 1), 34% in the moderate to severe disability category (1 ⩽ SHAQ < 2) and 7% in the severe to very severe disability category (2 ⩽ SHAQ ⩽ 3). The means of the visual analogue scales scores were in order of magnitude: overall disease severity (37 mm), RP (31 mm), pulmonary symptoms (24 mm), gastrointestinal symptoms (20 mm) and digital ulcers (19 mm). In multiple regression, the main factors associated with high SHAQ scores were the presence of dyspnoea [modified New York Heart Association (NYHA) class IV (regression coefficient B = 0.62), modified NYHA class III (B = 0.53) and modified NYHA class II (B = 0.21; all vs modified NYHA class I)], FM (B = 0.37), muscle weakness (B = 0.27), digital ulcers (B = 0.20) and gastrointestinal symptoms (oesophageal symptoms, B = 0.16; stomach symptoms, B = 0.15; intestinal symptoms, B = 0.15). Conclusion: SSc patients perceive dyspnoea, pain, digital ulcers, muscle weakness and gastrointestinal symptoms as the main factors driving their level of disability, unlike physicians who emphasize objective measures of disability.

Published
2017

30. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis

Author

Silvia Bellando-Randone, Mirko Manetti, Marco Matucci-Cerinic, Serena Guiducci, Eloisa Romano, Lidia Ibba-Manneschi, Irene Rosa, Amato de Paulis, Manetti, Mirko, Romano, Eloisa, Rosa, Irene, Guiducci, Serena, Bellando Randone, Silvia, DE PAULIS, Amato, Ibba Manneschi, Lidia, and Matucci Cerinic, Marco

Subjects
Male, Serum, 0301 basic medicine, CD31, Pathology, Mice, 0302 clinical medicine, Fibrosis, Immunology and Allergy, Endothelial dysfunction, Cells, Cultured, Skin, Mice, Knockout, integumentary system, Fibroblasts, Qualitative research, Systemic Sclerosis, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Cadherins, Platelet Endothelial Cell Adhesion Molecule-1, Phenotype, medicine.anatomical_structure, Fibroblast, Myofibroblast, Type I collagen, medicine.medical_specialty, Epithelial-Mesenchymal Transition, Endothelium, Collagen Type I, General Biochemistry, Genetics and Molecular Biology, Transforming Growth Factor beta1, Bleomycin, 03 medical and health sciences, Matrix Metalloproteinase 12, medicine, Animals, Humans, S100 Calcium-Binding Protein A4, Epithelial–mesenchymal transition, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, Mesenchymal stem cell, Endothelial Cells, medicine.disease, Urokinase-Type Plasminogen Activator, Actins, Mice, Inbred C57BL, Disease Models, Animal, 030104 developmental biology, Case-Control Studies, Microvessels, Snail Family Transcription Factors, business
Abstract

ObjectiveSystemic sclerosis (SSc) features multiorgan fibrosis orchestrated predominantly by activated myofibroblasts. Endothelial-to-mesenchymal transition (EndoMT) is a transdifferentiation by which endothelial cells (ECs) lose their specific morphology/markers and acquire myofibroblast-like features. Here, we determined the possible contribution of EndoMT to the pathogenesis of dermal fibrosis in SSc and two mouse models.MethodsSkin sections were immunostained for endothelial CD31 or vascular endothelial (VE)-cadherin in combination with α-smooth muscle actin (α-SMA) myofibroblast marker. Dermal microvascular ECs (dMVECs) were prepared from SSc and healthy skin (SSc-dMVECs and H-dMVECs). H-dMVECs were treated with transforming growth factor-β1 (TGFβ1) or SSc and healthy sera. Endothelial/mesenchymal markers were assessed by real-time PCR, immunoblotting and immunofluorescence. Cell contractile phenotype was assayed by collagen gel contraction.ResultsCells in intermediate stages of EndoMT were identified in dermal vessels of either patients with SSc or bleomycin-induced and urokinase-type plasminogen activator receptor (uPAR)-deficient mouse models. At variance with H-dMVECs, SSc-dMVECs exhibited a spindle-shaped appearance, co-expression of lower levels of CD31 and VE-cadherin with myofibroblast markers (α-SMA+ stress fibres, S100A4 and type I collagen), constitutive nuclear localisation of the EndoMT driver Snail1 and an ability to effectively contract collagen gels. Treatment of H-dMVECs either with SSc sera or TGFβ1 resulted in the acquisition of a myofibroblast-like morphology and contractile phenotype and downregulation of endothelial markers in parallel with the induction of mesenchymal markers. Matrix metalloproteinase-12-dependent uPAR cleavage was implicated in the induction of EndoMT by SSc sera.ConclusionsIn SSc, EndoMT may be a crucial event linking endothelial dysfunction and development of dermal fibrosis.

Published
2017

31. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

Author

Maurer, B., Graf, N., Michel, B. A., Muller Ladner, U., Czirjak, L., Denton, C. P., Tyndall, A., Metzig, C., Lanius, V., Khanna, D., Distler, O., Arner, I. H., Cerinic, M. M., Guiducci, S., Walker, U., Lapadula, G., Iannone, F., Becvar, R., Sierakowsky, S., Bielecka, O. K., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Vettori, S., Riemekasten, G., Rednic, S., Nicoara, I., Kahan, A., Allanore, Y., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Carreira, P. E., Novak, S., Varju, C., Chizzolini, C., Kucharz, E. J., Kotulska, A., Kopec Medrek, M., Widuchowska, M., Cozzi, F., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Hij, A., Airo, P., Hesselstrand, R., Scheja, A., Wollheim, F., Martinovic, D., Gurman, A. B., Braun Moscovici, Y., Govoni, Marcello, LO MONACO, Andrea, Hunzelmann, N., Pellerito, R., Bambara, L. M., Caramaschi, P., Black, C., Damjanov, N., Santamaria, V. O., Heitmann, S., Krasowska, D., Seidel, M., Oleszowsky, M., Burkhardt, H., Himsel, A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Starovoytova, M. N., Ananieva, L. P., Scorza, R., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szucs, G., Mendoza, A. Z., Buijdos, C. d. l. P., Sifuentes Giraldo, W. A., Midtvedt, O., Garen, T., Hachulla, E., Launay, D., Valesini, G., Riccieri, V., Ionescu, R. M., Opris, D., Groseanu, L., Wigley, F. M., Mihai, C. M., Cornateanu, R. S., Ionitescu, R., Gherghe, A. M., Gorga, M., Dobrota, R., Bojinca, M., Schett, G., Distler, J. H., Meroni, P., Zeni, P., Mouthon, L., Keyser, F. D., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Szechinski, J., Wiland, P., Szmyrka Kaczmarek, M., Sokolik, R., Morgiel, E., Krummel Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anic, B., Baresic, M., Mayer, M., Radominski, S. C., Muller, C. d. S., Azevedo, V. F., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Zenone, T., Highton, J., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Pisarri, S., Tanaseanu, C. M., Popescu, M., Dumitrascu, A., Tiglea, I., Chirieac, R., Ancuta, C., Furst, D. E., Kafaja, S., Lefebvre, P. G. d. l. P., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Kerzberg, E., Montoya, F., Cosentino, V., Chizzolini, Carlo, Maurer, Britta, Graf, Nicole, Michel, Beat A, Müller Ladner, Ulf, Czirják, László, Denton, Christopher P, Tyndall, Alan, Metzig, Carola, Lanius, Vivian, Khanna, Dinesh, Distler, Oliver, Tarner, Ingo H, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriele, Rednic, Simona, Nicoara, Ileana, Kahan, André, Allanore, Yannick, Vlachoyiannopoulos, P, Montecucco, Carlomaurizio, Caporali, Roberto, Carreira, Patricia E, Novak, Srdan, Varju, Cecilia, Kucharz, Eugene J, Kotulska, Anna, Kopec Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Airò, Paolo, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Gurman, Alexandra Balbir, Braun Moscovici, Yolanda, Govoni, M, Monaco, Andrea Lo, Hunzelmann, Nicola, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthia, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J, Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N, Ananieva, Lidia P, Scorza, Raffaella, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Mendoza, Antonio Zea, Buijdos, Carlos de la Puente, Giraldo, Walter A. Sifuente, Midtvedt, Øyvind, Garen, Torhild, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Mihai, Carmen M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Cantatore, Francesco P, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R, Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C, Müller, Carolina de Souza, Azevedo, Valderílio F, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Dougla, O’Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina Mihaela, Popescu, Monica, and University of Zurich

Subjects
Genetics and Molecular Biology (all), Male, Time Factors, Databases, Factual, systemic sclerosis, 2745 Rheumatology, computer.software_genre, Biochemistry, Severity of Illness Index, Outcomes Research, Qualitative Research, Systemic Sclerosis, Adult, Cohort Studies, Creatine Kinase, Decision Support Techniques, Deglutition Disorders, Dyspnea, Female, Fibrosis, Humans, Logistic Models, Middle Aged, Multivariate Analysis, Scleroderma, Diffuse, Sex Factors, Skin, Synovitis, Disease Progression, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Immunology and Allergy, Medicine (all), Scleroderma, skin fibrosis, skin and connective tissue diseases, ddc:616, EUSTAR, Univariate analysis, Database, integumentary system, 10051 Rheumatology Clinic and Institute of Physical Medicine, Orvostudományok, Diffuse, Connective tissue disease, Cohort, 2723 Immunology and Allergy, Cohort study, medicine.medical_specialty, 610 Medicine & health, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, NO, outcomes research, qualitative research, Databases, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, medicine, Factual, 2403 Immunology, business.industry, medicine.disease, business, computer
Abstract

ObjectivesTo identify predictive parameters for the progression of skin fibrosis within 1 year in patients with diffuse cutaneous SSc (dcSSc).MethodsAn observational study using the EUSTAR database was performed. Inclusion criteria were dcSSc, American College of Rheumatology (ACR) criteria fulfilled, modified Rodnan skin score (MRSS) ≥7 at baseline visit, valid data for MRSS at 2nd visit, and available follow-up of 12±2 months. Worsening of skin fibrosis was defined as increase in MRSS >5 points and ≥25% from baseline to 2nd visit. In the univariate analysis, patients with progressive fibrosis were compared with non-progressors, and predictive markers with pResultsA total of 637 dcSSc patients were eligible. Univariate analyses identified joint synovitis, short disease duration (≤15 months), short disease duration in females/patients without creatine kinase (CK) elevation, low baseline MRSS (≤22/51), and absence of oesophageal symptoms as potential predictors for progressive skin fibrosis. In the multivariate analysis, by employing combinations of the predictors, 17 models with varying prediction success were generated, allowing cohort enrichment from 9.7% progressive patients in the whole cohort to 44.4% in the optimised enrichment cohort. Using a second validation cohort of 188 dcSSc patients, short disease duration, low baseline MRSS and joint synovitis were confirmed as independent predictors of progressive skin fibrosis within 1 year resulting in a 4.5-fold increased prediction success rate.ConclusionsOur study provides novel, evidence-based criteria for the enrichment of dcSSc cohorts with patients who experience worsening of skin fibrosis which allows improved clinical trial design.

Published
2014

32. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative

Author

Patricia Carreira, Murray Baron, Sergio A. Jimenez, Madelon C. Vonk, Gabriele Valentini, James R. Seibold, Serena Guiducci, Peter A. Merkel, Christopher P. Denton, Bashar Kahaleh, Ariane L. Herrick, Marco Matucci-Cerinic, David H. Collier, Mary Ellen Csuka, Yannick Allanore, Sindhu R Johnson, Frank H J van den Hoogen, Daniel E. Furst, John Varga, Robert W. Simms, Alan Tyndall, Philip J. Clements, Armando Gabrielli, Douglas J. Veale, Stanislav Sierakowski, Janet E. Pope, Ulf Müller-Ladner, Jacob M van Laar, Ulrich A Walker, Barri J. Fessler, Virginia D. Steen, Vivien Hsu, László Czirják, Thomas A. Medsger, Otylia Kowal-Bielecka, Maureen D. Mayes, Gabriela Riemekasten, Murat Inanc, Jaap Fransen, Dinesh Khanna, Raymond P. Naden, Oliver Distler, Lorinda Chung, Richard M. Silver, Van Den Hoogen, Frank, Khanna, Dinesh, Fransen, Jaap, Johnson, Sindhu R., Baron, Murray, Tyndall, Alan, Matucci Cerinic, Marco, Naden, Raymond P., Thomas A., Medsger J. r., Carreira, Patricia E., Riemekasten, Gabriela, Clements, Philip J., Denton, Christopher P., Distler, Oliver, Allanore, Yannick, Furst, Daniel E., Gabrielli, Armando, Mayes, Maureen D., Van Laar, Jacob M., Seibold, James R., Czirjak, Laszlo, Steen, Virginia D., Inanc, Murat, Kowal Bielecka, Otylia, Müller Ladner, Ulf, Valentini, Gabriele, Veale, Douglas J., Vonk, Madelon C., Walker, Ulrich A., Chung, Lorinda, Collier, David H., Csuka, Mary Ellen, Fessler, Barri J., Guiducci, Serena, Herrick, Ariane, Hsu, Vivien M., Jimenez, Sergio, Kahaleh, Bashar, Merkel, Peter A., Sierakowski, Stanislav, Silver, Richard M., Simms, Robert W., Varga, John, Pope, Janet E., Matucci-Cerinic, Marco, Medsger Jr., Thomas A., Kowal-Bielecka, Otylia, Müller-Ladner, Ulf, University of Zurich, and van den Hoogen, Frank

Subjects
Male, 2745 Rheumatology, Acr criteria, Telangiectasi, 2736 Pharmacology (medical), Immunology and Allergy, Pharmacology (medical), Familial Primary Pulmonary Hypertension, skin and connective tissue diseases, integumentary system, Skin thickening, Diagnosis-Related Group, Interstitial lung disease, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Connective tissue disease, Autoantibodie, Europe, 2723 Immunology and Allergy, Evaluation of complex medical interventions Auto-immunity, transplantation and immunotherapy [NCEBP 2], Female, Human, United State, Adult, musculoskeletal diseases, medicine.medical_specialty, Consensus, Hypertension, Pulmonary, Immunology, Reproducibility of Result, 610 Medicine & health, Consensu, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Article, Rheumatology, Point system, 1300 General Biochemistry, Genetics and Molecular Biology, Scleroderma, Limited, Internal medicine, medicine, Humans, Telangiectasis, Disease Activity, Diagnosis-Related Groups, Systemic Sclerosi, Aged, Autoantibodies, 2403 Immunology, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Systemic, business.industry, Reproducibility of Results, Raynaud Disease, medicine.disease, United States, Treatment, Evaluation of complex medical interventions [NCEBP 2], Expert opinion, Physical therapy, business, Lung Diseases, Interstitial, Rheumatism
Abstract

Objective The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. Methods Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc. Results It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc. Conclusion The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease. Copyright © 2013 by the American College of Rheumatology.

Published
2013

33. Evidence for caveolin-1 as a new susceptibility gene regulating tissue fibrosis in systemic sclerosis

Author

Gilles Chiocchia, Philippe Dieudé, Marco Matucci-Cerinic, Serena Guiducci, Ilaria Tinazzi, Lidia Ibba-Manneschi, Cinzia Fatini, Giovanna Cuomo, Jean-Luc Cracowski, Eloisa Romano, Roberto Caporali, Maria Martinez, Vanessa Cohignac, Yannick Allanore, Eric Hachulla, Paola Caramaschi, Jean-Charles Lambert, Valeria Riccieri, Philippe Amouyel, Alessandra Della Rossa, Rosanna Abbate, Mohamad Saad, Paolo Airò, Luc Letenneur, Kiet Tiev, Mirko Manetti, Guido Valesini, Manetti, Mirko, Allanore, Yannick, Saad, Mohamad, Fatini, Cinzia, Cohignac, Vanessa, Guiducci, Serena, Romano, Eloisa, Airó, Paolo, Caramaschi, Paola, Tinazzi, Ilaria, Riccieri, Valeria, Della Rossa, Alessandra, Abbate, Rosanna, Caporali, Roberto, Cuomo, Giovanna, Valesini, Guido, Dieudé, Philippe, Hachulla, Eric, Cracowski, Jean Luc, Tiev, Kiet, Letenneur, Luc, Amouyel, Philippe, Lambert, Jean Charle, Chiocchia, Gille, Martinez, Maria, Ibba Manneschi, Lidia, and Matucci Cerinic, Marco

Subjects
Male, Fibrosi, Caveolin 2, Caveolin 1, Genome-wide association study, genome-wide association, scleroderma, pathogenesis, locus, disease, Linkage Disequilibrium, Genotype, Systemic sclerosis, genetics, caveolin-1, Medicine, Immunology and Allergy, skin and connective tissue diseases, Skin, education.field_of_study, integumentary system, Medicine (all), Chromosome Mapping, Middle Aged, Connective tissue disease, Female, Human, Adult, Population, European Continental Ancestry Group, Immunology, Single-nucleotide polymorphism, Polymorphism, Single Nucleotide, General Biochemistry, Genetics and Molecular Biology, White People, Rheumatology, SNP, Humans, Genetic Predisposition to Disease, Allele, education, Aged, Scleroderma, Systemic, Biochemistry, Genetics and Molecular Biology (all), business.industry, medicine.disease, Fibrosis, Minor allele frequency, business, Genome-Wide Association Study
Abstract

Objective Caveolin-1 (CAV1) is an inhibitor of tissue fibrosis and has been implicated in the pathogenesis of systemic sclerosis (SSc). The aim of the study was to analyse the possible association of CAV1 gene single nucleotide polymorphisms (SNP) with SSc. Methods A total population of 3974 individuals (1355 SSc patients, 2619 controls) was studied. Genotype data for 23 SNP spanning the CAV1–CAV2 gene locus were obtained from a genome-wide scan conducted in a French population (564 SSc patients, 1776 controls). Three CAV1 SNP (rs926198, rs959173, rs9920) displaying the most significant associations with SSc and/or clinical phenotypes were then genotyped in an Italian population (791 SSc patients, 843 controls). CAV1 protein expression in skin biopsies was investigated by immunohistochemistry and western blotting. Results In the French population, the CAV1 rs959173 C minor allele showed a significant protective association with susceptibility to SSc (OR 0.71, 95% CI 0.59 to 0.86, p adjusted =0.009), and with the subset of patients with limited cutaneous SSc (OR 0.71, 95% CI 0.56 to 0.89, p adjusted =0.018). The association was replicated in the Italian population and strengthened in the combined populations through Cochran–Mantel–Haenszel meta-analysis (SSc: pooled OR 0.81, 95% CI 0.71 to 0.92, p=0.0018; limited cutaneous SSc: pooled OR 0.80, 95% CI 0.69 to 0.93, p=0.0053). Genotype/protein expression correlations revealed that the rs959173 C protective allele was associated with increased CAV1 protein expression. Conclusions These results add CAV1 to the list of SSc susceptibility genes and provide further evidence for the contribution of this pathway in the fibrotic process that characterises SSc pathogenesis.

Published
2012

34. The Italian observational study on severe osteoporosis (ISSO): 24-month results on incidence of fractures and adherence to treatment

Author

Idolazzi, L., Maugeri, D., Monti, S., Massarotti, M., Osella, G., Barbagallo, M., Del Fiacco, R., Silvestri, S., Adami, S., Altomonte, L., Bardoscia, A., Bertoldo, F., Bevilacqua, M., Bianchi, G., Brancati, A., Cagnoni, C., Cantatore, F. P., Capone, A., Costanzo, G., D Avola, G., Giorgi, G., Di Matteo, L., Di Munno, O., Filipponi, P., Frisina, N., Fusco, A., Giannini, S., Guiducci, S., Iolascon, G., Isaia, G., Lombardi, G., Malavolta, N., Marcocci, C., Migliaccio, S., Migliore, A., Muratore, M., Nardi, A., Ortolani, S., Pasquali, R., Petto, H., Pietrogrande, L., Pola, E., Previti, B., Resmini, G., Alessandro Rubinacci, Russo, E., Scillitani, A., Silveri, F., Leali, P. T., Trotta, F., Ulivieri, M., Verdoia, C., Versace, F., Vinicola, V., Idolazzi, Luca, Maugeri, Domenico, Monti, Salvatore, Massarotti, Marco, Osella, Giangiacomo, Barbagallo, Mario, Del Fiacco, Romano, Silvestri, Sandra, Adami, Silvano, Altomonte, Lorenzo, Bardoscia, Alfredo, Bertoldo, Francesco, Bevilacqua, Maurizio, Bianchi, Gerolamo, Brancati, Annamaria, Cagnoni, Carlo, Cantatore, Francesco Paolo, Capone, Antonio, Costanzo, Giuseppe, D'Avola, Giovanni, De Giorgi, Giuseppe, Di Matteo, Luigi, Di Munno, Ombretta, Filipponi, Paolo, Frisina, Nicola, Fusco, Alessandra, Giannini, Sandro, Guiducci, Serena, Iolascon, Giovanni, Isaia, Giancarlo, Lombardi, Gaetano, Malavolta, Nazzarena, Marcocci, Claudio, Migliaccio, Silvia, Migliore, Alberto, Muratore, Maurizio, Nardi, Alfredo, Ortolani, Sergio, Pasquali, Renato, Petto, Helmut, Pietrogrande, Luca, Pola, Enrico, Previti, Baldassarre, Resmini, Giuseppina, Rubinacci, Alessandro, Russo, Enzo, Scillitani, Alfredo, Silveri, Ferdinando, Leali, Paolo Tranquilli, Trotta, Francesco, Ulivieri, Massimo, Verdoia, Cesare, Versace, Francesco, and Vinicola, Vincenzo

Subjects
Spinal fracture, Fracture, Rheumatology, Observational study, Osteoporosis therapy, Teriparatide, Immunology, Osteoporosi, Immunology and Allergy, Parathyroid hormone, Spine
Abstract

Objective To estimate the proportion of patients with very severe osteoporosis (those covered by the reimbursement criteria of the Italian National Health Service) experiencing new vertebral and non-vertebral fragility fractures in the first 24 months of a new anti-osteoporosis treatment. Methods Prospective observational study in men and post-menopausal women (aged > 21 years) initiating anti-osteoporosis treatment for very severe osteoporosis. Eligibility was based on teriparatide (TPD) reimbursement criteria in Italy: Incident of vertebral or hip fracture during anti-resorptive treatment (minimum 1 year), or at least three prevalent severe vertebral fractures, or two prevalent severe vertebral fractures and a historical proximal hip fracture. Incidence of new clinical vertebral and non-vertebral fractures was documented by original x-rays and/or radiological reports, and a post-hoc analysis compared data from the TPD monotherapy population versus the total treated group. Results Overall, 767 patients (mean age 72.8 years, 90.7% women) were enrolled in the study, of whom 628, 538, 419 and 424 attended visits at 6, 12, 18 and 24 months, respectively. The most commonly prescribed therapy was TPD (single-agent; 64.5%), then bisphosphonates and other anti-resorptives (33.3%). A combination of different oral treatments was given to 22.5% of the patients. Overall treatment adherence at 24 months was 65.7%. In a post-hoc analysis, the overall incidence of new clinical vertebral and non-vertebral fractures in the total treated population was, respectively, 4.7% and 2.3% in the first 6 months; 1.8% and 1.6% in the 6-12 month period; 2.9% and 1.4% in the 12-18 month period; and 2.2% and 1.0% in the 18-24 month period. Conclusion In patients with very severe osteoporosis, the risk of new vertebral and non-vertebral fractures declined after the first 6 months and remained low throughout the study.

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