Search

Your search keyword '"Lepri, G"' showing total 273 results
Start Over Author "Lepri, G"
273 results on '"Lepri, G"'

2. POS0044 DECIPHERING THE GUT MICROBIOTA OF VERY EARLY SYSTEMIC SCLEROSIS (VEDOSS) PATIENTS: A TAXONOMIC AND FUNCTIONAL CHARACTERIZATION

Author

Russo, E., primary, Lepri, G., additional, Baldi, S., additional, Fioretto, B. S., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Ghezzi, G., additional, Gloria, L. DI, additional, Orlandi, M., additional, Bertorello, S., additional, Bruni, C., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional

Published
2024
Full Text
View/download PDF

3. The Updated Role of Ultrasound in Assessing Dermatological Manifestations in Systemic Sclerosis

Author

Ruaro B, Santiago T, Hughes M, Lepri G, Poillucci G, Baratella E, Salton F, and Confalonieri M

Subjects
systemic sclerosis, ultrasound, elastosonography, skin, skin thickness, digital ulcers, Diseases of the musculoskeletal system, RC925-935
Abstract

Barbara Ruaro,1 Tania Santiago,2,3 Michael Hughes,4 Gemma Lepri,5 Gabriele Poillucci,6 Elisa Baratella,6 Francesco Salton,1 Marco Confalonieri1 1Unit of Pulmonology, University Hospital of Trieste, Trieste, Italy; 2Department of Rheumatology, Centro Hospitalare Universitário de Coimbra, Coimbra, Portugal; 3Medicine Faculty, University of Coimbra, Coimbra, Portugal; 4Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK; 5Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, Florence, Italy; 6Department of Radiology, Department of Medicine, Surgery and Health Science, University of Trieste, Trieste, ItalyCorrespondence: Barbara RuaroUnit of Pulmonology, University Hospital of Trieste, Trieste, ItalyTel +39 3470502394Email barbara.ruaro@yahoo.itAbstract: Systemic sclerosis (SSc), an autoimmune connective tissue disease, characterized by skin fibrosis, increased dermal thickness and microvascular involvement. Fibroblasts and myofibroblasts deposit excessive amounts of collagenous and non-collagenous extracellular matrix components in the skin. This leads to microvascular abnormalities and Raynaud’s phenomenon, with painful digital ulcers (DU) at the fingertips adding to patient discomfort. The skin involvement and severity in SSc was evaluated by the Modified Rodnan skin score (mRSS). Although high-frequency ultrasound (HUS) has been widely researched in the study of skin thickness and DU in SSc, its adoption into clinical practice is not yet common. However, novel insights into the still relatively unknown disease pathogenesis in SSc and its evaluation may be provided by HUS, including early (pre-clinical) skin involvement. It may also be useful in both the evaluation and follow-up of DU. Indeed, it is a non-invasive, safe, inexpensive and reproducible method able to assess not only SSc patients’ cutaneous structural changes, but also their vascular system changes. Moreover, several recent studies have reported that elastosonography (ES) is of use when investigating skin involvement in systemic sclerosis. This review aims at providing information as to role HUS and ES play in research advancements and the clinical perspectives in the evaluation of skin thickness and DU in SSc patients.Keywords: systemic sclerosis, ultrasound, elastosonography, skin, skin thickness, digital ulcers

Published
2021

4. 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, 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, 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, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, 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 S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., 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 M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., 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., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., 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, 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, 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, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, 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 S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., 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 M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., 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., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., and Rossella T.

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.

Published
2023

5. 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 S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., 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 M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., 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., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., 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, 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, 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, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, 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, S. L., Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., 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, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., 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., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., and Rossella, T.

Subjects
Scleroderma, Systemic, Settore MED/16 - REUMATOLOGIA, Epidemiology, Immunology, Systemic, Autoimmunity, Autoimmune Disease, Autoimmune Diseases, Scleroderma, Rheumatology, Immunology and Allergy, Seasons, Human
Abstract

ObjectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.MethodsData 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.ResultsAmong 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) (pConclusionThe 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.

Published
2023

7. 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, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, 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, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., Sebastiani G. D., De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, 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, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., and Sebastiani G. D.

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.

Published
2023

8. Lung vascular changes as biomarkers of severity in systemic sclerosis-associated interstitial lung disease

Author

Bruni, C., Occhipinti, M., Pienn, M., Camiciottoli, G., Bartolucci, M., Bosello, Silvia Laura, Payer, C., Balint, Z., Larici, Anna Rita, Tottoli, A., Tofani, L., De Lorenzis, E., Lepri, G., Bellando-Randone, S., Spinella, A., Giuggioli, D., Masini, F., Cuomo, G., Lavorini, F., Colagrande, S., Olschewski, H., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), Larici A. R. (ORCID:0000-0002-1882-6244), Bruni, C., Occhipinti, M., Pienn, M., Camiciottoli, G., Bartolucci, M., Bosello, Silvia Laura, Payer, C., Balint, Z., Larici, Anna Rita, Tottoli, A., Tofani, L., De Lorenzis, E., Lepri, G., Bellando-Randone, S., Spinella, A., Giuggioli, D., Masini, F., Cuomo, G., Lavorini, F., Colagrande, S., Olschewski, H., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), and Larici A. R. (ORCID:0000-0002-1882-6244)

Abstract

OBJECTIVES: It has recently become possible to assess lung vascular and parenchymal changes quantitatively in thoracic CT images using automated software tools. We investigated the vessel parameters of patients with SSc, quantified by CT imaging, and correlated them with interstitial lung disease (ILD) features. METHODS: SSc patients undergoing standard of care pulmonary function testing and CT evaluation were retrospectively evaluated. CT images were analysed for ILD patterns and total pulmonary vascular volume (PVV) extents with Imbio lung texture analysis. Vascular analysis (volumes, numbers and densities of vessels, separating arteries and veins) was performed with an in-house developed software. A threshold of 5% ILD extent was chosen to define the presence of ILD, and commonly used cut-offs of lung function were adopted. RESULTS: A total of 79 patients [52 women, 40 ILD, mean age 56.2 (s.d. 14.2) years, total ILD extent 9.5 (10.7)%, PVV/lung volume % 2.8%] were enrolled. Vascular parameters for total and separated PVV significantly correlated with functional parameters and ILD pattern extents. SSc-associated ILD (SSc-ILD) patients presented with an increased number and volume of arterial vessels, in particular those between 2 and 4 mm of diameter, and with a higher density of arteries and veins of <6 mm in diameter. Considering radiological and functional criteria concomitantly, as well as the descriptive trends from the longitudinal evaluations, the normalized PVVs, vessel numbers and densities increased progressively with the increase/worsening of ILD extent and functional impairment. CONCLUSION: In SSc patients CT vessel parameters increase in parallel with ILD extent and functional impairment, and may represent a biomarker of SSc-ILD severity.

Published
2023

9. POS0613 THE ROLE OF MICROBIOTA IN DIFFERENT DISEASE STAGES OF SYSTEMIC SCLEROSIS: A POSSIBLE IMPACT IN GASTROINTESTINAL TRACT DISEASE ACTIVITY?

Author

Russo, E., primary, Ghezzi, G., additional, Baldi, S., additional, Fioretto, B. S., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Lepri, G., additional, DI Gloria, L., additional, Bruni, C., additional, Melchiorre, D., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional

Published
2023
Full Text
View/download PDF

11. POS1313 EFFICACY OF IMMUNOGLOBULIN TREATMENT ON GASTROINTESTINAL, CUTANEOUS AND VASCULAR INVOLVEMENT IN SYSTEMIC SCLEROSIS: DATA FROM AN ITALIAN COHORT OF 65 PATIENTS

Author

Bonomi, F., primary, Damiani, A., additional, Coccia, C., additional, Levani, J., additional, Fiorentini, E., additional, Lepri, G., additional, Orlandi, M., additional, Bartoli, F., additional, Matucci-Cerinic, M., additional, Bellando-Randone, S., additional, and Guiducci, S., additional

Published
2023
Full Text
View/download PDF

12. Symposia

Author

Correll, Christoph, Kutcher, Stanley P., McClellan, John, Buitelaar, Jan, Pappadopulos, Elizabeth, Rothenberger, Aribert, Mattejat, Fritz, Scott, Stephen, Weisz, John, Schulz, Eberhard, Felder, Wilhelm, Fleischhaker, Christian, Böhme, R., Sixt, B., Jan van der Gaag, Rutger, Katz, Laurence Y., Cox, Brian J., Gunasekara, Shiny, Miller, Alec L., Laor, Nathaniel, Riedesser, Peter, Caffo, Ernesto, Leckman, James, Ammaniti, Massimo, Nicolais, Giampaolo, Speranza, Mario, Steiner, Hans, Delizonna, Laura, Schallauer, Astrid, Thienemann, Margo, McFarlane, Alexander C., van Hooff, Miranda, Sawyer, Michael, Cianchetti, Carlo, Gaddour, Naoufel, Sana, Mokni, Anouar, Mechri, Mondher, Letaief, Lotfi, Gaha, Härtling, Fabian, Bittner, Robert, Haenschel, Corinna, Cap, Marcus, Goncharova, Tanja, Linden, David E. J., Dittmann, Ralf, Maestele, Anneliese, Mehler, Claudia, Meyer, Eberhard, Jenner, Jack A., Boeing, Leonie, Murray, Val, Pelosi, Anthony, McCabe, Robert, Blackwood, Douglas, Wrate, Robert, Pellerano, S., Pintor, M., Mellis, G. L., Piroddi, T., Flisher, Alan, Nesa, Monique, Rooney, Rosanna, Roberts, Clare, Kane, Robert, Silburn, Sven, Pike, Lisbeth, Deaton, Helge Staby, Lustig, Stuart, Funk, Michelle, Rickards, Anne, Reddihough, Dinah, Wright-Rossi, Roslyn, Simpson, Jacqui, Seuthe, Dieter David, Vielhaber, H., Orden, Kinderklinik Dritter, Backmund, H., Gerlinghoff, M., Schwab-Stone, Mary, Jespers, Ine, Vermeiren, Robert, Ruchkin, Vladislav, Blatny, Marek, Hrdlicka, Michal, Urbanek, Tomas, Jelinek, Martin, Balastikova, Veronika, Jeammet, Philippe, Frottin, Alain, Filipovic, Andjelka, Albert, Eric, Schelotto, Dora Musetti, Knezevic, Mladen, Jovancevic, Milivoj, Hill, Jonathan, Lawlor, Maria, Kienbacher, Christian, Prause, Carolin, Stöckl, Margit, Bogyi, Gertrude, Friedrich, Max H., Klein, Michael, Kürschner, Katrin, Murray, Lynne, Leidecker, Victoria, Sharp, Helen, Luoma, Ilona, Kaukonen, Pälvi, Tamminen, Tuula, Nurcombe, Barry, Martin, Graham, McDermott, Brett, Resch, Franz, Schimmelman, Benno Graf, Edwards, Jane, McGorry, Patrick D., Lambert, Martin, Conus, Philippe, Preuss, Ulrich, Bürgin, Dieter, Strauss, Monika, Parzer, Peter, Spiel, Georg, von Korff, C., Ballin, H.-A., Gößler, R., Günter, M., Sange, G., Meng, Heiner, Koch, Eginhard, Minde, Klaus, True, Mary, Pisani, L., Oumar, F., Padilla, J., Bouville, Jean-François, Vogel, Wendy, Schmeck, Klaus, Goth, Kirstin, Purper-Ouakil, Diane, Dessons, Véronique, Doyen, Catherine, Perez-Diaz, Fernando, Mouren-Simeoni, Marie-Christine, Karwautz, Andreas, Wagner, Gudrun, Schwienbacher, Klaus, Haidvogl, Maria, Nobis, Gerald, Treasure, Janet Linda, Collier, David Andrew, Brunner, Romuald, Hueg, A., Haffner, Johann, Schmid, Marc, Goldbeck, Lutz, Nützel, Jakob, Höfling, Volkmar, Schermelleh-Engel, Karin, Moosbrugger, Helfried, Tomàs, Josep, Cornellà, Josep, Llusent, Alex, Bielsa, Anna, Belfer, Myron, Robertson, Brian, Mandlhate, Custodia, Seck, Birama, Zwirs, Barbara, Burger, Huib, Schulpen, Tom, Salman Al-Obedy, A. Karem, Romanchuk, Oleh, Namyslowska, Irena, Reigstad, Björn S., Jorgensen, Kirsti Margrethe, Matthys, Walter, Lochman, John, Zonnevylle-Bender, Marjo, van de Wiel, Nicolle, Wagner, Angela, Jennen-Steinmetz, Christine, Goepel, Christopher, van Bokhoven, Irene, van Goozen, Stephanie, Franciosi, L. Patt, Acquoy, Leode Graaf, Tischlinger, Anne, Pharm, B., Bronder, Knut Halyard, Schleimer, Kari, Walter, Joachim, Ephraime, Boia, Dmitrieva, Tatjana, Silva, Alvaro Seligman, Becker, Katja, Steinhausen, Hans-Christoph, Metzke, C. Winkler, Furtado, Erikson F., Laucht, Manfred, Bilke, Oliver, Zimmermann, Petra, Wittchen, Hans-Ulrich, Lieb, Roselind, Hannesdottir, Helga, Tyrfingsson, Thorarinn, Döpfner, Manfred, Hahlweg, Kurt, Kuschel, Annett, Bertram, Heike, Heinrichs, Nina, Freund-Braier, Inez, Brix, Gabriele, Hautmann, Christopher, Pluck, Julia, Crijnen, Alfons, van Lier, Pol, Vuijk, Patricia, Frank, Reiner, Vandvik, Inger Helene, Schäfert, Rainer, van Weel, Jeanne, Schieveld, Jan, Fegert, Jörg M., Friedrich, William, Celestin-Westreich, Smadar, Celestin, Leon Patrice, Ponjaert-Kristoffersen, Ingrid, Nagao, Keizo, Kisida, M., Shindo, E., Larsen, Helmer Baying, Helweg-Larsen, Karin, Lindauer, Ramón, Booij, Jan, Olff, Miranda, den Heeten, Gerard, Gersons, Berthold, Boer, Frits, Schoentjes, Eric, Bal, Sarah, Schulte-Markwort, Michael, Solantaus, Tytti, Toikka, Sini, Alasuutari, Maarit, Steck, Barbara, Grether, Andrea, Ehrensperger, M., Amsler, Felix, Kappos, L., Saha, Rina, Paschen, Bela, Baldus, Christiane, Haagen, Miriam, Pott, Martina, Romer, Georg, Ono, Yoshiro, Homma, H., Ishida, Y., Ide, H., Okamoto, M., Kameoka, S., Nakayama, Hiroshi, Yamamoto, A., Mukaddes, Nahit Motavalli, Tyano, Sam, Mozes, Tamar, Caplan, R., Malhotra, Savita, Ledda, Maria Giuseppina, Fratta, Al, Mannino, S., Corona, Simona, Zuddas, A., Olalla, Macarena Marin, Garcia, Ruth, Ramirez, Bernardo Perez, Campion, Ross, Hindley, Peter, Gupta, Nitin, Bhattacharaya, Anish, Kapoor, Mehak, van de Willge, G., Klemm, Silke, Smesny, S., Stockebrand, M., Grunwald, S., Juffer, Femmie, van Ijzendoorn, Marinus H., Bakermans-Kranenburg, Marian J., Ziegenhain, Ute, Derksen, B., Dreisörner, R., Gutschner, Daniel, Maldonado-Duran, Martin, Ferndndez-Criado, Manuel, Heidenreich, Felicia, Moro, Marie Rose, Millhuff, Charles, Pope, Kirby, Theisen, Frank, Himmerich, Hubertus, Kraus, T., Schuld, A., Pollmächter, T., Apter, Alan, Gothelf, D., Brand-Gothelf, A., Ratzoni, Gidi, Kikinzon, L., Weizman, A., Bloch, Yuval, Haberhausen, Michael, Müller, Daniel, Fayyad, John, Filho, Altino Bessa Marques, de Menezes, Adolfo Bezerra, Campo, John, Shafer, Sheree, Strohm, Jennifer, Lucas, Amanda, Shaeffer, David, Altman, Harold, Gelachek, Christine, Motomura, Naoyasu, Takino, Yozo, Iwakiri, Masahiro, Pössel, Patrick, Seemann, Simone, Hautzinger, Martin, Mutale, Theodore, Haase, Christian, Abidi, Majid Ali, Raheem, Shehla, Faw, Leyla, Hogue, Aaron, Liddle, Howard, Catthoor, Kirsten, Hutsebaut, Joost, Jasinski, Donald, Faries, Douglas, Moore, Rodney, Streeck-Fischer, Annette, Sannwald, Renate, Barth, Gottfried Maria, Schwarz, Christoph, Staigle, Monika, Pham, Manh-Hiep, Balanzin, Dario, Materi, Joelle, Eresund, Pia, Mokhovikov, Alexander, Stankovic, Sandra, Munir, Kerim, Erol, Nese, Çetin, Füsun Çuhadarodlu, Hassiotis, Angela, Flament, Martine, Scholz, Michael, Rix, Maud, Nestler, Franziska, Selisko, Annegret, Godart, Nathalie, Perdereau, Fabienne, Rein, Zoé, Curt, Florence, Akister, Jane, Lee, Pei-Chin Peggy, Tsai, Sho-Man Susan, Ho, Lai-Shiun, Wu, Su-Chun, Miermont, Jacques, Swenson, Joel, Teherani, Mardjane, Falissard, Bruno, Cottraux, Jean, Plück, Julia, Oades, Robert, Simons-Sprong, Mirjam, Schothorst, P. F., Swaab-Barneveld, J. T., Juran, Stephanie, Weisbrod, Matthias, Chen, Eric, Röpcke, Bernd, Popovic-Deusic, Smiljka, Poustka, Luise, Wild-Wall, Nele, Papousek, Mechthilde, Keren, Mirelle, Feldman, Ruth, Maestro, S., Chilosi, A., Pecini, C., Pfanner, L., Greenhill, Laurence, Jahnsen, K., den Berg, L. T. W. Jong-van, Zito, J. M., Posner, Kelly, Skrobala, Anne, Goldberg, Pablo, Kotler, Lisa, Findling, Robert, Bussing, Regina, Sayal, Kapil, Mitchell, Geoffrey, Huss, Michael, Högl, Barbara, Grimmlinger, Renate, Käppler, Karl Christoph, Teodoro, Maycoln M. L., Oswald, Sylvia Hiromi, Dagnoni, Janine M., Pinheiro, M. I., Heleno, C. T., Rothe-Neves, R., Haase, V. G., Prette, A. Del, Lambertucci, Marimilia Rodrigues, Rodrigues, J. L., Freitas, P. M., Lourenco, C. A. P., Carvalho, H. C. W., Baumeister, J., Weisenhorn, M., Stadelmann, S., Oswald, S. H., Ruder, H., Ruggerini, Ciro, Vicini, Stefania, Pupulin, Enrico, Guidi, Antonio, Puura, Kaija, Mäntymaa, Mirjami, von Klitzing, Kai, Rosvald, Orna, Kröber, Hans-Ludwig, Stöver, A., Proske, R., Semmelbeck, Rainer, Walther, Marc, Schmelzle, Matthias, Egli-Alge, Monika, Beckett, Richard, Gerhold, Constanze, Turkmen-Barta, Lieselotte, Chiland, Colette, Meyer-Bahlburg, Heino, Ceglie, Domenico Di, Lehmkuhl, Ulrike, Uccellini, Orlando, Bertolini, Mario, Neri, Francesca, Albanese, Delia, Bertola, Raffaella, Snoek, Maartje, Kas, Martien, Schulze, Ulrike Margarete Elisabeth, Calame, Silke, Keller, Ferdinand, Santel, Stephanie, Krauel, Kerstin, Rotte, Michael, Münte, Thomas F., van Elburg, Annemarie, Poustka, Fritz, Bölte, Sven, Feineis-Matthews, Sabine, Boite, Sven, Hubl, D., Prvulovic, D., Dierks, T., Klauck, Sabine, Moilanen, Irma, Mattila, M., Laurila, J., Jussila, K., Pyper, A., Linna, S. L., Ebeling, Hanna, Pauls, D., Korpilahti, Pirjo, Loukusa, Soile, Jansson-Verkasalo, Eira, Hebebrand, Johannes, Herpertz-Dahlmann, Beate, Hinney, Anke, Wermter, Anne-Kathrin, Friedel, Susann, Geller, Frank, Schafer, Helmut, Fernandez-Aranda, Fernando, Holliday, Joanna, Holtkamp, Kristian, Mika, C., Heer, M., Uher, Rudolf, Barbarich, Nicole, Henry, Shannan E., Bailer, Ursula, Frank, Guido, Kaye, Walter H., Wentz, Elisabet, Lacey, J. Hubert, Waller, Glenn, Rastam, Maria, Turk, Jeremy, Gillberg, Christopher, Verhulst, Frank C., Zwaanswijk, Marieke, Verhaak, Peter, Bensing, Jozien, van der Ende, Jan, Verhulst, Frank, Sourander, Andre, Santalahti, Paivi, Ford, Tamsin, Goodman, Robert, Meltzer, Howard, Seiffge-Krenke, Inge, Fritsch, Richard, Cutler, Marika, Anthony, E. James, Rydelius, Per-Anders, Castell, Rolf, Pejovic-Milovancevic, Milica, Pavlovic, Miroslav, Kalman, Noa, Linder, Muli, Luria, Ido, Levkovitz, Yechiel, Yamazaki, Kosuke, da Silva, Pedro Caldeira, Santos, Grata, Martins, Filipa, Chiu, Yen-Nan, Tsai, Wen-Jer, Gau, Shur-Fen, Tseng, Chang-Chang, Su, Shu-Chen, Croonenberghs, Jan, Brouw, Lucas, Wauters, Annick, Bruning, Nicole, Manjaly, Zina, Fink, Gereon, Aleksic, Olivera, Rudic, Nenad, Jansen, Lucres, Wied, Christine C. Gispen-De, Lahuis, Bertine, Swaab, Hanna, Pietersen, Jolijn, Gevers, Carolien, Kamp-Becker, Inge, Germerott, Isabell, Howlin, Patricia, Gaudière, Forresi, Barbara, Lepri, G., Laval, Soumaila, Wiefel, Andreas, Biringen, Z., Titze, Karl, Lenz, K., Seither, C., Witte, B., Dunitz-Scheer, Marguerite, Wilken, Markus, Krasnovsky, Alexandra, Scheer, Peter, Cordeiro, Maria, Muratori, Filippo, Felloni, B., Cesari, A., Helmig, Linda, Fonagy, Peter, Moody, Chris, Fultz, Jim, Glanzmann, René, Lutz-Latil, Nathalie, van Wyl, Agnes, Puras, Dainius, Hervds, Amaia, Tsiantis, John, Dragonas, T., Davis, H., Ispanovic, V., Paradisiotou, A., Shanini, Mimoza, Jones, Lynne, Uka, A., Rrustemi, A., von Knorring, Anne-Liis, Deboutte, Dirk, Dorhmi, Souraya, Agoub, Mohamed, Moussaoui, Driss, Battas, Omar, Halvorsen, Inger, Andersen, Anne, Heyerdahl, Sonja, Baillot, Denise, La Roche, Michele, Furino, Claudia, Buchholtz, Annick, Goldfield, Gary, Henderson, Katherine, Hagenah, Ulrich, Blume, Varinja, Flacke-Redanz, Marlene, Dahlmann, Beate Herpertz, Sallas, Angelique A., Adam, Hubertus, Ephraime, Boia, Jr., Mozambique, Goci-Uka, Aferdita, Schlüter-Müller, Susanne, Bawa, Umesh, Khalik, Fakhri, Forouher, Nima, Sadamatsu, Miyuki, Nanba, Kato, Nobumasa, Kasai, Kiyoto, Nanba, Eiji, Schmidt, Martin H., Esser, Günter, van Engeland, Herman, Willemsen-Swinkels, Sophie, Dietz, Claudine, Beernink, Anne Claire, Vidojevic, Oliver, Milacic, Ivona, Strous, Rael, Hegesh, Roni, Kertzman, Simion, Ben-Nahum, Z., Kotler, Moshe, van Daalen, Emma, Zeegers, Mijke, Pol, Hilleke Hulshoff, Williams, Charles, Sank, Jessica, Paulk, Martha, Schwarz, George, Wharton, Paul, Raleva, Marija, Paketchieva, Kamka, Filipovska, Angelina, Nix, Carole Müller, Guex, Margarita Forcada, Daigham, Abdel-Mohsen, Indredavik, Marit S., Vik, Torstein, Kulseng, Siri, Brubakk, Ann-Mari, Cuddy, Marion, Riley, Katharine, Vollmer, Brigitte, Wyatt, John, Murray, Robin, Soininen, Mika, Paavonen, E. Juulia, Fjällberg, Mika, Salmi, Juha, Fredrik, Almgvist, Aronen, Eeva T., Reis, Olaf, Bohne, Stephanie P., Kraenz, Susanne, Ahn, Dong-Hyun, Kim, Tae-Ho, Choi, Jun-Ho, Kim, Yun-Young, Begovac, Ivan, Skocic, Milena, Rudan, Vlasta, Filipovic, Oleg, Wolanczyk, Tomasz, Brynska, Anita, Wojtowicz, Stanislaw, Celia, Salvador, Aerts, Cisca, Cleve, Elisabeth, Hartmann, Hellmut, Kühle, Hans-Jürgen, Heidorn, Fridjof, Zeyer, Solveigh, Fuentes, Joaquin, Martin, Andrés, Sukhodolsky, Denis G., Kaltiala-Heino, Riittakerttu, Rimpelä, Matti, Andershed, Henrik, O’Donnell, Deborah, Pearce, Michelle, Burgin, Dieter, Becker, Andreas, Hagenberg, Nicola, Berking, Matthias, Roessner, Veit, Hanssen-Bauer, Ketil, Aalen, Odd, Junglas, Jürgen, Huh, Yoon-Seok, Kim, Yun Young, Oh, Kyung-Ja, Wang, Kai, Tarren-Sweeney, Michael, Leiblum, D. M., Kühl, Renate, Nötzel, Cornelia, Pfeiffer, Ernst, Lenz, Klaus, Rosling, Agneta, Poller, Marianne, Cross, Donna, Klabin, Simone, Kaplan, Diana, Mickel, Lars, Lehmkuhl, Gerd, Möckel, Regina, Leor, Shani, Frisch, Leor, Frisch, Amos, Weizman, Abraham, Zanozin, Andrey, Jamart, Sylvie, Hayez, Jean-Yves, Leor, Agnes, Ahle, Maria Elisabeth, Amitay, Galit Ben, Kosov, Irene, Reiss, Ahuva, Tamar, Moses, Smedje, Hans, Allik, Hiie, Steyaert, Jean, Castermans, Dries, Creemers, John, Kaczynska-Haladyj, Koenraad Devriendt Marta, Ballabriga, Maria Claustre Jané, Judez, Joaquima, Pelaez, Empar, Sole, Pilar, Rodriguez, Lidia, Palmen, Saskia, Kemner, Chantal, Schnack, Hugo, Kahn, Rene, Fabrizi, Anna, Gabriel, Levi, Mercadante, Marcos, Ramos, Sergiode Paula, Rosario-Campos, Maria Concecao, Rutter, Michael, Collishaw, Stephan, Maughan, Barbara, Pickles, Andrew, Messer, Julie, Caspi, Avshalom, Moffitt, Terrie, Kreppner, Jana, Borge, Anne Inger H., Luthar, Suniya, Hamarman, Stephanie, Ulger, C., Fossella, J., Brimacombe, M., Dermody, J., Stein, Mark, Waldman, L. D., Sarampote, C., Robb, A., Cook, E. H., Kirley, Aiveen, Lowe, N., Hawi, Z., Mullins, C., Daly, G., Waldman, I., McCarro, M., O’Donell, van der Meulen, Emma, Bakker, S. C., Pauls, D. L., Sinke, R. J., Polanczyk, Guilherme, Zeni, C., Genro, J. P., Roman, Tatiana, Hutz, Mara, Schaff, Christa, Haemmerle, Patrick, Sontag, Harald, Vetro, Agnes, Gadoros, Julia, Roosen-Runge, Gotthard, Hattab, Jocelyn, Hummel, Peter, Braun-Lewensohn, Orna, Schechter, Daniel, Zeanah, Charles, Myers, Michael, Liebowitz, Michael, Davies, Mark, Soong, Wei-Tsuen, James, Deborah, Sofroniou, Nick, Gegelashvili, Marine, Parikh, Umesh, Kane, John M., Malhotra, Anil K., Shah, Manoj, Pleak, Richard R., Hizami, Ronen, Michelson, David, Danckaerts, Marina, Zuddas, Alessandro, Zhang, Shuyu, Hazell, Philip, Zeiner, P., Johnson, M., Häßler, Frank, Suyash, Prasad, Sonuga-Barke, Edmund, Poole, Lynne, Mares, Sarah, Jureidini, Jon, Steel, Zachary, Newman, Louise, Lucas, Torsten, Paulus, Stephanie, Aßhauer, Martin, Miller, Birgit, Björn, Gunilla Jarkman, Bodén, Christina, Gustafsson, Per, Ivkic, Viola Povse, Tenjovic, Lazar, Jelena, Radosavljev, Deusic, Smiljka Popovic, Graham, Philip, Klasen, Henrikje, Tan, Jacinta, Hope, Tony, Stewart, Anne, Fitzpatrick, Raymond, Kölch, Michael, Diaz-Caneja, Angeles, Johnson, Sonia, Dippold, Ines, Keller, Katja Wiethoffi Ferdinand, Bailey, Sue, Whittle, Nathan, Hennighausen, Klaus, Kohls, Gregor, Maas, Verena, Rinker, Tanja, Zachau, Swantje, Christmann, Gabriele, Jaremkiewicz, Anna, Schecker, Michael, von Suchodoletz, Waldemar, Uwer, Ruth, Albrecht, Ronald, Glass, Lisa, Csépe, Valéria, Honbology, Ferenc, Rago, Anett, Mészdros, Eva, Schwartz, Richard G., Shafer, Valerie L., Green, Jonathan, Jacobs, C., Kroll, L., Briskman, J., Dunn, G., Beecham, J., Tobias, B., Baird, L., Ogden, Terje, Fitzgerald, Michael, Bellgrove, Mark, Gill, Michael, Robertson, Ian H., McArdle, Paul, Burke, Amanda, Hong, K. Michael, Hoven, Christina, Wasserman, Danuta, Braun, Katharina, Bock, Jórg, Helmecke, Carina, Gruß, Michael, Poeggel, Gerd, Marsden, Charles, Muchimapura, S., Pardon, M.-C., Bianchi, M., Feldon, Joram, Rüedi-Bettschen, Daniela, Dettling, Andrea C., Pryce, Christopher R., Clement, Hans-Willi, Sommer, O., Pschibul, A., Rombach, C., Gerlach, M., Mehler-Wex, Claudia, Zeiske, S., Grünblatt, E., Gille, G., Rausch, D., Gerlach, Manfred, El-Din, Amira Seif, Kadri, Nadia, Andaloussi, Houda Hjiej, Chihabeddine, Khadija, Almaqrami, Mohammed, von Gontard, Alexander, Okuno, M., Quaschner, Kurt, Bilenberg, Niels, Obel, Carsten, Henriksen, Tine Brink, Hedegaard, Morten, Secher, Niels Jurgen, Olsen, Jorn, Fonseca, Antonio, Koch, Isabelle Nathalie, Bite, Ieva, Cohen, Phyllis, Russell, Katrin, Broyden, Nichaela, Lancaster, Gillian, Eichhorn, Christina, Tiedtke, Karola, Feldman, Ronald, Warnke, Andreas, Scheuerpflug, Peter, Vetter, V., Bartling, Jürgen, Konrad, Kerstin, Neufang, Susanne, Hanisch, Charlotte, Fink, Gereon R., Durston, Sarah, Davidson, Matthew C., Tottenham, Nim, Spicer, Julie, Galvan, Adriana, Horvitz, John, Fossella, John A., Watts, Richard, Casey, B. J., Brandeis, Daniel, Fallgatter, Andreas J., Ehlis, Ann-Christine, Seifert, Jürgen, Strik, W. K., Zillessen, K. E., Herrmann, Martin J., Schulte-Körne, Gerd, Lyytinen, Heikki, Guttorm, Tomi, Poikkeus, Anna-Maija, Eklund, Kenneth M., Lyytinen, Paula, Torppa, Minna, Laakso, M.-L., Leskinen, E., Tolvanen, A., Paracchini, Silvia, Schumacher, J., König, I. R., Libertus, Claudia, Griesemann, Heide, Kleensang, A., Ziegler, A., Propping, P., Näthen, M., Wolmer, Leo, Zagout, Iyad, Galili-Weisstub, Esti, Fisch, Gene, Swillen, Ann, Vogels, Annick, Freitag, Christine, Bouville, Jean-Francois, Atlanti-Duault, Laetitia, Baubet, Thierry, Osrow, Robyn, Leplomb, Marie-Madeleine, Marchandy, Yves, Bennabi, Malika, Halpern, Ricardo, Monteiro, Odon, Durkin, Abbey-Robin, Haapanen, Rudy, Bauer, Susanne, Friedrich, Max, Stadler, Christina, Sterzer, Philipp, Kleinschmidt, Andreas, Nowraty, Irene, Müller, W. E., Knölker, Ulrich, Schmid, Gabriele, Berndt, Swantje, Behn, B., Puls, Jan Hendrik, Stevens, Luc, Jungmann, Joachim, Juretic, Zoran, Ercegovic, Nela, Schepker, Renate, Çuhadaroglu-Çetin, Füsun, Herhaus, G., Melfsen, S., Cheng, Daomeng, Harder, Donald, Laws, Harry, Nakane, Yoshibumi, Takeshita, Kenzo, Naruse, Hiroshi, Zhu, Yan, Liu, Jun, Du, Yasong, Sikorski, John, Hamerlynck, Sannie, Hart, Lisettet, Nauta-Janssen, Lucres, Chitsabesan, Prathiba, Nguyen, Hien, Simeon, Jovan, Cuzner, Cathy, Schachter, Howard, Martins, Ana Soledade, Kieling, Chirstian, Comassetto, Julia, Goncalves, Renata, Oswald, Silvia, Buchmann, Johannes, Kirschner, J., Garvey, M., Moll, Gunther, Heinrich, Hartmut, Malhotra, Sameer, Poulakis, Zeffie, Menahem, Sam, Sauer, Karin, Samia, Tilouch, Rimeh, Hannachi, Sonia, Missaoui, Allodi, Mara Westling, Biscaldi, Monica, Wagner, Bettina, Uchida, Chiyoko, Jozefiak, Thomas, Penge, Roberta, Biaggini, Valentina Ivancich, Fischbein, Siv, Joukamaa, Matti, Taanila, Anja, Veijola, Juha, Karvonen, Juha T., Miettunen, Jouko, Llaberia, Edelmira Domenech, Domenech, Teresa Corbella, Ballabriga, Maria Claustre Jane, Sanz, Josepa Canals, Esparo, Griselda, Sola, Sergi Ballespi, Liu, Xuejun, Kano, Yukiko, Ohta, Masataka, Nagai, Yoko, Arai, Takashi, Linyan, Su, Bridge, Jeff, Birmaher, Boris, Di Lorenzo, Carlo, Iyengar, Satish, Brent, David, Blanz, Bernhard, Weninger, Laura, Libal, Gerhard, Skrabal, Anna, Bowden, Michael, Cooper, Howard, Simonsen, Inger, Bechstrom, Carl, Medby, Mette, Erkolahti, Ritva, Klosinski, Gunther, Oba, Mihoko, Murase, Satomi, Murakami, Takashi, Takai, Jiro, Kaneko, Hitoshi, Honjo, Shuji, Rickards, Katrina, Weber, Annhild, Karle, Michael, Lazartigues, Alain, Planche, Pascale, Lemonnier, Eric, Pavuluri, Mani, Schenkel, Lindsay, Shaw, Ryan, Sweeny, John, Rigon, Giancarlo, Costa, Stefano, Mancaruso, Alessandra, Mansi, Roberta, Poggioli, Daniele Giovanni, Chiodo, Simona, Radobuljac, Maja, Groleger, Urban, Ovsenik, Nada, Tomori, Martina, Haas, Barbara, Denoix, Susanne, Kimmig, Franz, Weinhardt, Marc, Schmitz, Günter, Filschke, Berit, Fliegauf, Conny, Kim, Ji-Hae, Krischer, Maya, Stone, Michael H., Sevecke, Kathrin, Doepfner, Manfred, König, Cornelia, Grasmann, Dörte, Schlander, Michael, Ralston, Stephen, Pereira, R. Rodrigues, Brussel, W., Vlasveld, L., Tuynman-Qua, H. G., Lorenzo, M. J., Tauscher-Wisniewski, R., Palazzi, Stefano, Guaia, Ettore, Kolakowski, Artur, Pisula, Agnieszka, Wilens, Timothy, Banaschewski, Tobias, Uebel, Henrik, Albrecht, Björn, Robatzek, Monika, Migliaccio-Walle, Kristen, Caro, Jaime, Allen, Albert J., Sangal, R. Bart, Owens, Judith, Kelsey, Douglas, Sutton, Virginia, Schuh, Kory, Bahadir, Aliye Tugba, Yaman, Zeynep, Arman, Ayse Rodopman, Kuscu, Kemal, Yazgan, Yanki, Berkem, Meral, Feldman, Peter, Denai, Milton, Simpson, Alexander, Kratochvil, Christopher, Newcorn, Jeffrey, Biederman, Joseph, Gelowitz, Douglas, Thomason, Christine, Gao, Haitao, Bijttebier, Patricia, Decoene, Steff, Niklaus, Pia, Duits, Nils, Auer, Ulrich, Schnoor, Kathleen, Schläfke, Detlef, Çetin, Füsun Çuhadaroälu, Harper, Gordon, Hamdan, Sami, El-Haib, Muhammed, Canat, Saynur, Halfon, Olivier, Bolognini, Monique, Plancherel, B., Phan, Olivier, Corcos, Maurice, Cardinaux, Jean-René, Magistretti, Pierre J., Pierrehumbert, Blaise, Koskinen, Minna, Engqvist, Ulf, Allin, Matthew, Rifkin, Larry, Lancaster, Sandra, Borghini, Ayala, Jaugey, Laure, Forcada-Guex, Margarita, Jaunin, Lyne, Müller-Nix, Carole, Ansermet, François, Simoes, Mariada Conceicao Taborda, Lima, Luiza Nobre, Dias, Mariada Luz Vale, Siefen, Rainer Georg, Como, Ariel, Alikaj, Valbona, Tomori, Sonila, Capozzi, Flavia, Romano, Angela, Roello, Mara, Piperno, Francesca, Mann, Mali A., Stösser, Dieter, Barth, Gottfried, Pimenov, Alina, Schwab, Jenny, Bingöl, Hülya, Barbe, Rémy, Elkshishy, Heba, Jovanovic, Ana, Lakic, Aneta, Milovanovic, Vesna, Vukasinovic, Milorad, Bridge, Jeffrey, Kolko, David, Brent, David A., Gilson, Kathryn, Montague, Roslyn, Shochet, Ian, Marques, Cristina Maria Ribeiro, Cepeda, Teresa, Ligges, Carolin, Ligges, Marc, Huonker, Ralph, Leppänen, Paavo, Guttorm, Torni K., Hämäläinen, Jarmo, Puolakanaho, Anne, Plume, Ellen, König, Inke R., Deimel, Wolfgang, Nöthen, Markus M., Propping, Peter, Kleensang, André, Müller-Myhsok, Bertram, Ziegler, Andreas, Hong, Sung-Do David, Gao, Xueping, Li, Xuerong, Lee, Soyoung Irene, Kim, Eui-Jung, Cho, In-Hee, Kim, Ji-Hoon, Park, Se-Hyun, Choi, J-Wook, Heger, Steffen, Schreiner, Andreas, Rettig, Klaus, Medori, Rossella, Gustafsson, Peik, Hansson, Kjell, Eidevall, Lena, Thernlund, Gunilla, Heiser, Philip, Dempfle, A., Smidt, Judith, Grabarkiewicz, Justyna, Kiefl, Hans, Hemminger, U., Saar, K., Swanson, James, Volkov, Nora D., Gupta, S., Williams, L., Agler, D., Wasdell, M., Wigal, S., Martins, Silvia, Tramontina, Silza, Eizirik, Mariana, Vitiello, Benedetto, Clevenger, Walter, Faraone, Stephen, McGough, James, McCracken, James, Rohde, Luis Augusto, Greenhill, Larry, Leary, Michael, Larsson, Bo, Gunning, W. Boudewijn, Villat, Jean-Marie, O’Connell, Redmond, Bellgrove, Mark A., Dockree, Paul, Traube, Raymond, Braunschweig, Mary, Chabanier, Jacques, De Leo, Germana, Ibanez, Margerita, Mikolajaks, Olivette, Ropstad, Ida, Young, J. Gerald, Aiello, Rachele, Porcari, Viviana, Salatiello, Maria Patrizia, Lo Bue, Anna, Dell’Oglio, Valentina, Cardella, Rosaria, Chifari, Sabrina, Undheim, Anne Mari, Su, Linyan, Luo, Xuerong, Barton, Joanne, Baying, Lioba, Rellum, Thomas, Duezel, Emrah, Hinrichs, Hermann, Bartel, Christoph, Linde, Iris, Friederichs, E., Bangs, Mark, Remschmidt, Helmut, Doreleijers, Theodore, Rebernig, Elisabeth, Camerini, Giovanni Battista, Otero, Soraya, Rivas, Ana, Pombo, Guadalupe, Yeghiyan, Maruke, Kachatur, Gasparyan, Danileyan, Arman, Ivarsson, Tord, Valderhaug, Robert, Walitza, Susanne, Wewetzer, Christoph, Barth, Nikolaus, Hahn, F., Asbahr, Fernando, Castillo, Ana Regina, Ito, Ligia, Latorre, Mariado Rosario, Moreira, Michelle, Lotufo-Neto, Francisco, Symann, Sophie, Charlier, Dominique, Plattner, Belinda, Schallauer, Astrid Elisabeth, Mohler, Beat, Staub, P., Müller, Carsten, Oelkers-Ax, Rieke, Fischer, Jochen, Hermanns, Uta, Nickel, Anne, Bolay, Hans Volker, Cherro-Aguerre, Miguel, Sorensen, Merete Juul, Nissen, Judith Becker, Mors, Ole, Thomsen, Per Hove, Sund, Anne Mari, Drugli, May Britt, Wichstrom, Lars, Schwannauer, Matthias, Taylor, Emily, Wrate, Rob, Martin, Matthias, Larsson, Jan-Olov, Larsson, Henrik, Lichtenstein, Paul, Ludolph, Andrea G., Mottaghy, Felix, Kraemer, Susanne, Claus, Dieter, Krause, Bernhard, Fegert, Jbrg M., Hurtig, Tuula, Malakhova, Anna, Maniadaki, Katerina, Kakouros, Efthymios, Jensen, Peter, Garcia, Joe Albert, Glied, Sherry, Crowe, Maura, Foster, E. Michael, Golse, Bernard, Junghanß, Jenny, Salin, Aino-Maija, Rytölä, Päivi, Hiltunen, Pauliina, Remschmidt, Helmut, editor, and Belfer, Myron L., editor

Published
2004
Full Text
View/download PDF

14. Prognostic Value of Lung Ultrasound B-Lines in Systemic Sclerosis

Author

Gargani, L., Bruni, C., Romei, C., Frumento, P., Moreo, A., Agoston, G., Guiducci, S., Bellando-Randone, S., Lepri, G., Belloli, L., Della Rossa, A., Delle Sedie, A., Stagnaro, C., De Nes, M., Salvadori, S., Mosca, M., Falaschi, F., Epis, O., Picano, E., Matucci-Cerinic, M., and Gargani, Luna

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, systemic sclerosis, Population, Critical Care and Intensive Care Medicine, Pulmonary function testing, B-lines, 03 medical and health sciences, 0302 clinical medicine, DLCO, Internal medicine, medicine, In patient, 030212 general & internal medicine, education, interstitial lung disease, education.field_of_study, ultrasound, business.industry, Ultrasound, Interstitial lung disease, prognosis, medicine.disease, Rheumatology, Lung ultrasound, 030228 respiratory system, Radiology, Cardiology and Cardiovascular Medicine, business
Abstract

Background A high percentage of systemic sclerosis (SSc) patients experience interstitial lung disease (ILD) during the disease course. Recent data have shown that lung ultrasound (LUS) can assess ILD by the evaluation of B-lines, the sonographic sign of pulmonary interstitial involvement. Research Question To establish the prognostic value of B-lines in a large number of patients with SSc. Study Design and Methods A total of 396 consecutive patients with SSc, who were enrolled at three Rheumatology Departments, underwent a comprehensive LUS examination on the anterolateral and posterior chest for a total of 58 scanning sites. All available clinical, imaging, and functional data were recorded. Patients were followed after enrolment to establish the prognostic role of LUS. Results The median number of B-lines was higher in patients with the diffuse cutaneous subset (44 vs 17 B-lines; P Interpretation Lung ultrasound B-lines are associated with worsening or development of pulmonary deterioration. In the near future, LUS might become part of the diagnostic and prognostic armamentarium in patients with SSc, which would allow a more sustainable and user-friendly approach to this very fragile population.

Published
2020
Full Text
View/download PDF

15. POS0487 THE CROSSTALK OF THE SKIN-ORAL-GUT MICROBIOME AXIS IN LIMITED AND DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS

Author

Russo, E., primary, Carboni, D., additional, Baldi, S., additional, Fioretto, B., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Lepri, G., additional, DI Gloria, L., additional, Bruni, C., additional, Melchiorre, D., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional

Published
2022
Full Text
View/download PDF

16. AB0747 Oral microbiome in rheumatic diseases.What involvement?

Author

Ceccherini, M. T., primary, Bellando-Randone, S., additional, Guiducci, S., additional, Romano, E., additional, Carboni, D., additional, El Aoufy, K., additional, Lepri, G., additional, Cometi, L., additional, Tofani, L., additional, Matucci-Cerinic, M., additional, and Melchiorre, D., additional

Published
2022
Full Text
View/download PDF

18. Undifferentiated connective tissue disease: State of the art on clinical practice guidelines

Author

Antunes, M, Scire, C, Talarico, R, Alexander, T, Avcin, T, Belocchi, C, Doria, A, Franceschini, F, Galetti, I, Govoni, M, Hachulla, E, Launay, D, Lepri, G, Macieira, C, Matucci-Cerinic, M, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Paolino, S, Ramoni, V, Tani, C, Tas, S, Tincani, A, Van Vollenhoven, R, Zen, M, Fonseca, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Beretta, L, Antunes M., Scire C. A., Talarico R., Alexander T., Avcin T., Belocchi C., Doria A., Franceschini F., Galetti I., Govoni M., Hachulla E., Launay D., Lepri G., Macieira C., Matucci-Cerinic M., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Paolino S., Ramoni V., Tani C., Tas S. W., Tincani A., Van Vollenhoven R., Zen M., Fonseca J., Bombardieri S., Fonseca J. E., Schneider M., Smith V., Cutolo M., Mosca M., Beretta L., Antunes, M, Scire, C, Talarico, R, Alexander, T, Avcin, T, Belocchi, C, Doria, A, Franceschini, F, Galetti, I, Govoni, M, Hachulla, E, Launay, D, Lepri, G, Macieira, C, Matucci-Cerinic, M, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Paolino, S, Ramoni, V, Tani, C, Tas, S, Tincani, A, Van Vollenhoven, R, Zen, M, Fonseca, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Beretta, L, Antunes M., Scire C. A., Talarico R., Alexander T., Avcin T., Belocchi C., Doria A., Franceschini F., Galetti I., Govoni M., Hachulla E., Launay D., Lepri G., Macieira C., Matucci-Cerinic M., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Paolino S., Ramoni V., Tani C., Tas S. W., Tincani A., Van Vollenhoven R., Zen M., Fonseca J., Bombardieri S., Fonseca J. E., Schneider M., Smith V., Cutolo M., Mosca M., and Beretta L.

Abstract

The term a € undifferentiated connective tissue disease' (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.

Published
2019

21. POS0317 THE PERFORMANCE OF DIFFUSING CAPACITY FOR MONOXIDE CARBON (DLCO) AND FORCED VITAL CAPACITY (FVC) IN PREDICTING THE ONSET OF SYSTEMIC SCLEROSIS (SSc)-INTERSTITIAL LUNG DISEASE (ILD) IN THE EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) DATABASE

Author

Lepri, G., primary, Bruni, C., additional, Tofani, L., additional, Moggi Pignone, A., additional, Orlandi, M., additional, Sara, T., additional, Hughes, M., additional, Del Galdo, F., additional, Irace, R., additional, Distler, O., additional, Riccieri, V., additional, Allanore, Y., additional, Gheorghiu, A. M., additional, Siegert, E., additional, De Vries-Bouwstra, J., additional, Hachulla, E., additional, Tikly, M., additional, Damjanov, N., additional, Spertini, F., additional, Mouthon, L., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Guiducci, S., additional, Matucci-Cerinic, M., additional, Furst, D., additional, and Bellando Randone, S., additional

Published
2021
Full Text
View/download PDF

23. OP0181 FUNCTIONAL CUT-OFFS TO DISTINGUISH PULMONARY VASCULAR AND PARENCHYMAL INVOLVEMENT IN SYSTEMIC SCLEROSIS (SSC): A QUANTITATIVE ANALYSIS OF IMAGING FEATURES AT CHEST COMPUTED TOMOGRAPHY (CT)

Author

Bruni, C., primary, Occhipinti, M., additional, Camiciottoli, G., additional, Bartolucci, M., additional, Lepri, G., additional, Fabbrizzi, A., additional, Tottoli, A., additional, Bassetto, A., additional, Ciardi, G., additional, Giuggioli, D., additional, Cuomo, G., additional, Masini, F., additional, Lavorini, F., additional, Calistri, L., additional, and Matucci-Cerinic, M., additional

Published
2020
Full Text
View/download PDF

25. THU0360 EFFICACY OF A SELF-TREATMENT PROTOCOL FOR FACE AND TEMPOROMANDIBULAR JOINTS REHABILITATION IN SYSTEMIC SCLEROSIS (SSC)

Author

Passalacqua, M., primary, Foggi, C., additional, Mauro, N., additional, Tofani, L., additional, Guiducci, S., additional, Bruni, C., additional, Lepri, G., additional, Blagojevic, J., additional, El Aoufy, K., additional, Fiori, G., additional, Bartoli, F., additional, Maddali Bongi, S., additional, Mitola, M., additional, Gizduloch, M., additional, Matucci-Cerinic, M., additional, and Bellando Randone, S., additional

Published
2020
Full Text
View/download PDF

26. AB0550 DIFFUSING CAPACITY OF THE LUNG FOR CARBON MONOXIDE (DLCO) VS FORCED VITAL CAPACITY (FVC): SYSTEMATIC LITERATURE REVIEW AND META-ANALYSIS TO EXAMINE THEIR ABILITY TO MEASURE CHANGE IN CLINICAL TRIALS IN SYSTEMIC SCLEROSIS (SSC)

Author

Bellando Randone, S., primary, Cappellini, E., additional, Nidiaci, L., additional, Lepri, G., additional, Lazzaroni, M. G., additional, Campochiaro, C., additional, Bagnato, G., additional, Sambataro, D., additional, Sambataro, G., additional, Matucci-Cerinic, M., additional, and Furst, D., additional

Published
2020
Full Text
View/download PDF

27. SAT0553 QUANTITATIVE ANALYSIS OF IMAGING FEATURES AT CHEST CT OF PULMONARY ARTERIAL AND VENOUS COMPONENTS IN SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE (SSc-ILD).

Author

Bruni, C., primary, Occhipinti, M., additional, Camiciottoli, G., additional, Bartolucci, M., additional, Pienn, M., additional, Lepri, G., additional, Fabbrizzi, A., additional, Tottoli, A., additional, Ciardi, G., additional, Giuggioli, D., additional, Cuomo, G., additional, Masini, F., additional, Olschewski, H., additional, Lavorini, F., additional, Calistri, L., additional, and Matucci-Cerinic, M., additional

Published
2020
Full Text
View/download PDF

28. Systemic sclerosis: State of the art on clinical practice guidelines

Author

Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., Matucci-Cerinic M., Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., and Matucci-Cerinic M.

Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and nonpharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

Published
2018

29. Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Author

Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Lepri, G, Jaeger, Vk, Walker, Ua, Iannone, F, Cacciapaglia, F, Tomčík, M, Becvar, R, Rednic, S, Petcu, A, Szabo, I, Codullo, V, Caporali, R, Montecucco, C, Carreira, P, Ioven, B, Minier, T, Czirják, L, Chizzolini, C, Allali, D, Zanatta, E, Doria, A, Gabrielli, A, Airò, P, Lazzaroni, Mg, Radić, M, Martinovic, D, Braun-Moscovici, Y, Balbir-Gurman, A, Hunzelmann, N, Caramaschi, P, Morovic-Vergles, J, Denton, C, Santamaria, V, Heitmann, S, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Foeldvari, I, Helmus, N, Salvador, M, Stamenkovic, B, Stankovic, A, Ananieva, L, Herrick, A, Engelhart, M, De La Puente, C, Hoffmann-Vold, Am, Midtvedt, Ø, Launay, D, Sobanski, V, Riccieri, V, Opris-Belinski, D, Groseanu, L, Ionescu, R, Bojinca, M, Sunderkötter, C, Distler, J, Ingegnoli, F, van der Haecke, A, Ullman, S, Pozzi, Mr, Eyerich, K, Vanthuyne, M, Erler, A, Aringer, M, De Langhe, E, Baresic, M, Mayer, M, Anic, B, Yavuz, S, Granel, B, Popa, S, Agachi, S, Zenone, T, Mathieu, A, Vacca, A, Solanki, K, Veale, D, Loyo, E, Tineo, C, Gigante, A, Rosato, E, Oksel, F, Yagurcu, F, Tănăseanu, Cm, Visalli, E, Benenati, A, Foti, R, Ancuta, C, Dan, D, Adler, S, Villiger, P, Fathi, N, de la Peña Lefebvre PG, González Martín, J, Chatelus, E, Sibilia, J, Litinsky, I, Del Galdo, F, Ann Sakettkoo, L, Kerzberg, E, Bianchi, Wa, Bianchi, Bv, Castellví, I, Limonta, M, Rimar, D, Couto, M, Ribi, C, Spertini, F, Kahl, S, Hsu, V, Poindron, V, Meghit, K, Martin, T, Kolstad, K, Chung, L, Thiele, A, Schmeiser, T, Zdrojewski, Z, Riemekasten, G, Levy, Y, Cardoneanu, A, Burlui, A, Rezus, E, Pamuk, On, Talotta, R, Bongiovanni, S, Puttini, Ps., Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Giovanna, Cuomo, Chizzolini, Carlo, Allali, Danièle, and University of Zurich

Subjects
Adult, Male, medicine.medical_specialty, Databases, Factual, systemic sclerosis, digital ulcer, 610 Medicine & health, Disease, ddc:616.07, Logistic regression, Systemic scleroderma, Cohort Studies, Rheumatology, Risk Factors, Internal medicine, Cox proportional hazards regression, medicine, Humans, Pharmacology (medical), In patient, digital ulcers, gangrene, vasculopathy, Aged, Gangrene, Scleroderma, Systemic, business.industry, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, food and beverages, Middle Aged, medicine.disease, Cross-Sectional Studies, Cohort, Female, business, systemic sclerosi
Abstract

Objective In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

Published
2019

31. Agricultural cooperative 'Co.r.ag.gio.', an example of urban agriculture in Rome (Italy) as a resilient strategy against urban climate change

Author

MARICCHIOLO, FRIDANNA, PANNO, ANGELO, CARRUS, GIUSEPPE, Lepri, G., Brizi, A., N. Kabisch, J, Stadler, S. Duffield, H. Korn, A. Bonn, Maricchiolo, Fridanna, Lepri, G., Brizi, A., Panno, Angelo, and Carrus, Giuseppe

Abstract

In urban contexts, cultivated agricultural land increases permeability, improves positive atmospheric exchanges, protects the complexity of agricultural ecosystem and helps general resilience to urban climate changes. We present the Italian case study of the EU-FP7 funded project called “GLAMURS - Green Lifestyles, alternative models and upscaling regional sustainability” (www.glamurs.eu). GLAMURS investigates transitions to sustainable lifestyles and green economies, through an interdisciplinary approach in seven different regions of Europe. In the Lazio Region of Italy, the young agricultural cooperative "Co.R.Ag.Gio" (“Courage”), COoperativa Romana AGricoltura GIOvani (Roman Agricultural Cooperative of Youth), encourages citizens and institutions to conserve environmental heritages abandoned in the Roman agricultural outskirts. CoRAgGio was founded in 2011, as a free association of young people (farmers, agronomists, chefs, architects, day workers, industrial worker, anthropologists, educators). It promotes an original work perspective in this economic crisis, enhancing passions and experiences in agricultural and horticultural activities, teaching and training (educational farms, courses in sustainable agriculture), food (spreading good practices, 0-km production), crafts. It strives to make public land available to all citizens, and preserve agricultural soils from the expansion of the building sector. It promotes an agricultural urban model that is healthy, organic, multi-functional, replacing the degraded concrete buildings with a proposal of a new way of living, based on ecological concerns, respecting labour dignity, and social meanings of agriculture. The implications of promoting sustainable urban agriculture will be discussed, in terms of economic values, ecological services and food production, improvement of life quality, soil protection, earth resources and biodiversity conservation.

Published
2017

32. Clinical-serological characterization and treatment outcome of a large cohort of Italian children with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection and Pediatric Acute Neuropsychiatric Syndrome.

Author

Lepri, G, Rigante, Donato, Bellando Randone, S, Meini, A, Ferrari, A, Tarantino, Giusyda, Cunningham, Mw, Falcini, F, Rigante D (ORCID:0000-0001-7032-7779), Tarantino G, Lepri, G, Rigante, Donato, Bellando Randone, S, Meini, A, Ferrari, A, Tarantino, Giusyda, Cunningham, Mw, Falcini, F, Rigante D (ORCID:0000-0001-7032-7779), and Tarantino G

Abstract

Objective: Pediatric autoimmune neuropsychiatric disorder associated with Streptococcus pyogenes infection (PANDAS) and pediatric acute-onset neuropsychiatric syndrome (PANS) are emerging immune-mediated encephalopathies characterized by sudden onset of seemingly inexplicable complex neuropsychiatric symptoms, including obsessions, compulsions, and heterogeneous tics, which occur in children. Main goal of this study was to report our experience in a large cohort of Italian children affected by either PANDAS or PANS and treated long term with an antibiotic regimen similar to that used for acute rheumatic fever. Patients and Methods: The clinical charts of a cohort of 371 consecutive Italian children, 345 with PANDAS (93.0%) and 26 with PANS (7.0%), were retrospectively evaluated. Antistreptococcal, antinuclear antibodies, and serologic evaluation for a group of common autoantibodies and microbial agents were also assessed. A strict differential diagnosis with other autoimmune diseases displaying neuropsychiatric manifestations was performed. Results: Antistreptolysin O and anti-DNase B antibody titers were tested and were positive in all PANDAS subjects, but negative in PANS. Anti-Mycoplasma pneumoniae antibodies and anti-Epstein-Barr virus Nuclear Antigen antibodies were found positive in 11 (42.3%) and 5 (19.2%) patients with PANS, respectively. Among PANDAS cases, a clear streptococcal infection was clinically evident at the onset of neurological symptoms in only 74 patients (21.4%), whereas the relationship with Streptococcus pyogenes was confirmed by serologic tests in the other 271 (78.6%). All patients fulfilling the diagnostic criteria for PANDAS (n = 345) received amoxicillin/clavulanic acid for 10-21 days at diagnosis, while those who were diagnosed with PANS (n = 26) received treatment according to the causative agent. Thereafter, all PANDAS/PANS patients received prophylaxis with benzathine benzylpenicillin for an overall period of at least 5 years to pre

Published
2019

33. One year in review 2018: systemic vasculitis

Author

Baldini, C., Quartuccio, L., Talarico, R., Posarelli, C., Calabresi, E., Felicetti, M., Cavallaro, E., Lepri, G., Sara Monti, Bond, M., and Elefante, E.

Subjects
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antirheumatic Agents, Churg-Strauss Syndrome, Cryoglobulinemia, Giant Cell Arteritis, Granulomatosis with Polyangiitis, Hepatitis C, Chronic, Humans, Immunosuppressive Agents, Microscopic Polyangiitis, Systemic Vasculitis, Takayasu Arteritis, Hepatitis C, Chronic
Abstract

Systemic vasculitis are heterogeneous, complex and disabling disorders. Following the previous annual reviews of this series, this paper gives a brief overview on current knowledge about recent literature on small- and large-vessel systemic vasculitis, with a specific focus on pathogenetic and clinical aspects, novel possible disease-related biomarkers and current and future therapies that are in the pipeline.

Published
2018

34. THU0425 18f-fluorodeoxyglucose positron emission tomography/computed tomography and lung involvement in systemic sclerosis

Author

Bellando Randone, S., primary, Tartarelli, L., additional, Cavigli, E., additional, Tofani, L., additional, Bruni, C., additional, Lepri, G., additional, Blagojevic, J., additional, Moggi Pignone, A., additional, Mihai, C., additional, Avouac, J., additional, Passeri, A., additional, De Cristofaro, M.T., additional, Distler, O., additional, Allanore, Y., additional, Guiducci, S., additional, and Matucci Cerinic, M., additional

Published
2018
Full Text
View/download PDF

35. Da Grande Farò il Pompiere: il ruolo dei genitori nelle scelte di formazione Universitaria

Author

Nota, L, Soresi, S, Camussi, E, Gritti, A, Annovazzi, C, Frasca, C, Patrizi, P, Lepri G., L, Lodi, E, Bussu, A, CAMUSSI, ELISABETTA, GRITTI, ALICE, ANNOVAZZI, CHIARA, Lepri G. , L, Bussu, A., Nota, L, Soresi, S, Camussi, E, Gritti, A, Annovazzi, C, Frasca, C, Patrizi, P, Lepri G., L, Lodi, E, Bussu, A, CAMUSSI, ELISABETTA, GRITTI, ALICE, ANNOVAZZI, CHIARA, Lepri G. , L, and Bussu, A.

Published
2015

36. Systemic sclerosis: State of the art on clinical practice guidelines

Author

Smith, V. (Vanessa), Scirè, C.A. (Carlo Alberto), Talarico, R. (Rosaria), Airo, P. (Paolo), Alexander, T. (Tobias), Allanore, Y. (Yannick), Bruni, C. (Cosimo), Codullo, V. (Veronica), Dalm, V.A.S.H. (Virgil), Vries-Bouwstra, J.K. (Jeska) de, Della Rossa, A. (Alessandra), Distler, O. (Oliver), Galetti, I. (Ilaria), Launay, D. (David), Lepri, G. (Gemma), Mathian, A. (Alexis), Mouthon, L. (Luc), Ruaro, B. (Barbara), Sulli, A. (Alberto), Tincani, A. (Angela), Vandecasteele, E. (Els), Vanhaecke, A. (Amber), Vanthuyne, M. (Marie), Hoogen, F.H.J. van den, Van Vollenhoven, R.F. (Ronald F.), Voskuyl, R.A. (Robert), Zanatta, E. (Elisabetta), Bombardieri, S. (Stefano), Burmester, G.R. (Gerd), Eurico, F.J. (Fonseca João), Frank, C. (Charissa), Hachulla, E. (Eric), Houssiau, F. (Frederic), Mueller-Ladner, U. (Ulf), Schneider, M. (Matthias), Van Laar, J.M. (Jacob M), Vieira, A. (Ana), Cutolo, M. (Maurizio), Mosca, M. (Marta), Matucci-Cerinic, M. (Marco), Smith, V. (Vanessa), Scirè, C.A. (Carlo Alberto), Talarico, R. (Rosaria), Airo, P. (Paolo), Alexander, T. (Tobias), Allanore, Y. (Yannick), Bruni, C. (Cosimo), Codullo, V. (Veronica), Dalm, V.A.S.H. (Virgil), Vries-Bouwstra, J.K. (Jeska) de, Della Rossa, A. (Alessandra), Distler, O. (Oliver), Galetti, I. (Ilaria), Launay, D. (David), Lepri, G. (Gemma), Mathian, A. (Alexis), Mouthon, L. (Luc), Ruaro, B. (Barbara), Sulli, A. (Alberto), Tincani, A. (Angela), Vandecasteele, E. (Els), Vanhaecke, A. (Amber), Vanthuyne, M. (Marie), Hoogen, F.H.J. van den, Van Vollenhoven, R.F. (Ronald F.), Voskuyl, R.A. (Robert), Zanatta, E. (Elisabetta), Bombardieri, S. (Stefano), Burmester, G.R. (Gerd), Eurico, F.J. (Fonseca João), Frank, C. (Charissa), Hachulla, E. (Eric), Houssiau, F. (Frederic), Mueller-Ladner, U. (Ulf), Schneider, M. (Matthias), Van Laar, J.M. (Jacob M), Vieira, A. (Ana), Cutolo, M. (Maurizio), Mosca, M. (Marta), and Matucci-Cerinic, M. (Marco)

Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and nonpharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

Published
2018
Full Text
View/download PDF

37. Cross-sectional evaluation of plasma vitamin D levels in a large cohort of Italian patients with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection

Author

Stagi, S, Lepri, G, Rigante, Donato, Matucci Cerinic, M, Falcini, F., Rigante D (ORCID:0000-0001-7032-7779), Stagi, S, Lepri, G, Rigante, Donato, Matucci Cerinic, M, Falcini, F., and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

BACKGROUND: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) are immune-mediated diseases characterized by obsessive-compulsive symptoms and/or tics triggered by group A Streptococcus infections. Despite the well-known action of 25-hydroxyvitamin D [25(OH)D] on different conditions driven by systemic inflammation, there are no data about the 25(OH)D status in patients with PANDAS. AIMS: To evaluate plasma 25(OH)D levels in a large cohort of children and adolescents with PANDAS and comparing the results with healthy controls. METHODS: We have evaluated plasma 25(OH)D levels in 179 Italian patients with PANDAS (49 females, 130 males, mean age at diagnosis: 101.4 ± 30.1 months) and in an age-, gender-, and body mass index-matched control group of 224 healthy subjects. RESULTS: Patients with PANDAS have shown more frequently reduced 25(OH)D levels (<30 ng/mL) in comparison with controls (94.6% vs. 82.5%, p = 0.0007). Patients with PANDAS had also lower levels of 25(OH)D than controls (20.4 ± 6.9 ng/mL vs. 24.8 ± 7.3 ng/mL, p < 0.0001). This difference was observed during both winter (13.7 ± 3.25 ng/mL vs. 21.4 ± 5.9 ng/mL, p < 0.0001) and summer (21.8 ± 6.5 ng/mL vs. 32.5 ± 8.7 ng/mL, p < 0.0001). Notably, serum 25(OH)D levels correlated with both number of streptococcal (strep) infections before diagnosis of PANDAS (p < 0.005) and with infection recurrence (p < 0.005). CONCLUSIONS: PANDAS patients have reduced 25(OH)D levels, which appear related to streptococcal infections and the probability of recurrence. Further long-term studies with higher number of patients are needed to investigate and confirm this relationship.

Published
2018

42. Effects of rituximab in connective tissue disorders related interstitial lung disease

Author

Lepri, G., Avouac, J., Airò, P., Santos, F. A., Bellando-Randone, S., Blagojevic, J., Hernàndez, F. G., Gonzalez Nieto, J. A., Guiducci, S., Jordan, S., Limaye, V., Maurer, B., Selva-O Callaghan, A., Valeria RICCIERI, Distler, O., Matucci-Cerinic, M., Allanore, Y., University of Zurich, and Allanore, Yannick

Subjects
2403 Immunology, 2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, 610 Medicine & health, skin and connective tissue diseases
Published
2016

47. Preliminary analysis of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis

Author

Minier, T., Guiducci, S., Bellando-Randone, S., Bruni, C., Lepri, G., Czirjak, L., Distler, O., Walker, U. A., Fransen, J., Allanore, Y., Denton, C., Cutolo, M., Tyndall, A., Muller-Ladner, U., Matucci-Cerinic, M., Airo, P., Zingarelli, S., Ananieva, L., Desinova, O., Ancuta, C. M., Belibou, C. I., Avouac, J., Becvar, R., Skacelova, S., Beretta, L., Vigone, B., Caramaschi, P., Sabbagh, D., Carpentier, P., Damjanov, N., Simic-Pasalic, K., Distler, J. H. W., Farge-Bancel, D., Hadj-Khelifa, S., Foti, R., Di Gangi, M., De La Pena Lefebvre, P. G., Hachulla, E., Salvador, M. J., Kayser, C., Camargo, C. Z., Kumanovics, G., Li, M., Xu, D., Marasini, B., Belloli, L., Maurer, B., Mayer, M., Mihai, C., Gherghe, A. M., Riccieri, V., Stefanantoni, K., Salsano, F., Rosato, E., Senecal, J. -L., Koenig, M., Senet, P., Frances, C., Sipek, A., Stankovic, A., Stamenkovic, B., Smith, V., Tarner, I. H., Wiland, P., University of Zurich, and Matucci-Cerinic, Marco

Subjects
Male, Anti-nuclear antibody, Pulmonary Fibrosis, 2745 Rheumatology, Scleroderma, Microscopic Angioscopy, Cohort Studies, Antinuclear, Immunology and Allergy, skin and connective tissue diseases, integumentary system, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Connective tissue disease, Autoantibodies, Disease Activity, Outcomes research, Systemic Sclerosis, Adult, Antibodies, Antinuclear, Early Diagnosis, Female, Fingers, Humans, Raynaud Disease, Scleroderma, Systemic, Skin Ulcer, Telangiectasis, Cohort, 2723 Immunology and Allergy, musculoskeletal diseases, medicine.medical_specialty, Immunology, 610 Medicine & health, General Biochemistry, Genetics and Molecular Biology, Antibodies, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, 2403 Immunology, business.industry, Systemic, Autoantibody, medicine.disease, Surgery, stomatognathic diseases, Inflammatory diseases Radboud Institute for Health Sciences [Radboudumc 5], Observational study, business, Rheumatism
Abstract

Item does not contain fulltext OBJECTIVES: The EULAR (European League Against Rheumatism) Scleroderma Trials and Research Group (EUSTAR) has identified preliminary criteria for very early diagnosis of systemic sclerosis (SSc). Our aim was to assess the prevalence of each proposed diagnostic item in a large observational patient cohort with Raynaud's phenomenon (RP). METHODS: Baseline data of 469 RP patients enrolled into the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) cohort are presented. RESULTS: 68% of all RP patients were antinuclear antibody (ANA) positive. ANA+ RP patients more frequently had previous or current puffy fingers (PuFi) (38.5% and 23.3%, p

Published
2014

48. Severe vitamin D deficiency in patients with Kawasaki disease: a potential role in the risk to develop heart vascular abnormalities?

Author

Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Falcini, F., Rigante, Donato (ORCID:0000-0001-7032-7779), Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Falcini, F., and Rigante, Donato (ORCID:0000-0001-7032-7779)

Abstract

Twenty-five-hydroxyvitamin D (25(OH)-vitamin D) is crucial in the regulation of immunologic processes, but-although its deficiency has been reported in patients with different rheumatological disorders-no data are available for Kawasaki disease (KD). The goals of this study were to assess the serum levels of 25(OH)-vitamin D in children with KD and evaluate the relationship with the eventual occurrence of KD-related vascular abnormalities. We evaluated serum 25(OH)-vitamin D levels in 79 children with KD (21 females, 58 males, median age 4.9 years, range 1.4-7.5 years) in comparison with healthy sex-/age-matched controls. A significantly higher percentage of KD patients (98.7 %) were shown to have reduced 25(OH)-vitamin D levels (<30 ng/mL) in comparison with controls (78.6 %, p < 0.0001). Furthermore, KD patients had severely low levels of 25(OH)-vitamin D than controls (9.17 ± 4.94 vs 23.3 ± 10.6 ng/mL, p < 0.0001), especially the subgroup who developed coronary artery abnormalities (4.92 ± 1.36 vs 9.41 ± 4.95 ng/mL, p < 0.0001). In addition, serum 25(OH)-vitamin D levels correlated not only with erythrosedimentation rate (p < 0.0001), C-reactive protein (p < 0.0001), hemoglobin level at KD diagnosis (p < 0.0001) but also with both coronary artery aneurysms (p = 0.005) and non-aneurysmatic cardiovascular lesions (p < 0.05). Low serum concentrations of 25(OH)-vitamin D might have a contributive role in the development of coronary artery complications observed in children with KD.

Published
2016

49. Vitamin D receptor polymorphisms are not associated with the risk of Kawasaki disease (KD) in a group of Italian children

Author

Falcini, F, Marini, F, Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Brandi, Ml, Rigante, Donato (ORCID:0000-0001-7032-7779), Falcini, F, Marini, F, Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Brandi, Ml, and Rigante, Donato (ORCID:0000-0001-7032-7779)

Abstract

This description deals with the evaluation of a relationship between vitamin D receptor polymorphisms in a group of Italian children and the risk to develop Kawasaki disease, the most important vasculitis in the pediatric age.

Published
2016

Catalog

Books, media, physical & digital resources
See catalog results