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1. Environment and Mesothelioma

Author

Di Maria, Giuseppe, primary, Bruno, Caterina, additional, Vancheri, Ada, additional, Di Maria, Chiara, additional, and Comba, Pietro, additional

Published
2022
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2. Histogram-based analysis in progressive pulmonary fibrosis: relationships between pulmonary functional tests and HRCT indexes

Author

Palmucci, Stefano, primary, Tiralongo, Francesco, additional, Galioto, Federica, additional, Toscano, Stefano, additional, Reali, Linda, additional, Scavone, Carlotta, additional, Fazio, Giulia, additional, Ferlito, Agata, additional, Sambataro, Gianluca, additional, Vancheri, Ada, additional, Sciacca, Enrico, additional, Vignigni, Giovanna, additional, Spadaro, Carla, additional, Mauro, Letizia Antonella, additional, Foti, Pietro Valerio, additional, Vancheri, Carlo, additional, and Basile, Antonio, additional

Published
2023
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5. Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet: the MADIET clinical trial

Author

Molina-Molina, Maria, primary, Shull, Jessica Germaine, additional, Vicens-Zygmunt, Vanesa, additional, Rivera-Ortega, Pilar, additional, Antoniou, Katerina, additional, Bonella, Francesco, additional, Renzoni, Elisabetta, additional, Russell, Anne-Marie, additional, Maher, Toby M., additional, Vancheri, Ada, additional, Bachs, Anna, additional, Avilés, Victoria, additional, Palma, Josep, additional, Bermudo, Guadalupe, additional, Suarez-Cuartin, Guillermo, additional, Tebé, Cristian, additional, Rigo-Bonnin, Raul, additional, Montes-Worboys, Ana, additional, Wijsenbeek, Marlies, additional, and Vancheri, Carlo, additional

Published
2023
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6. Assessment of Lung Cancer Development in Idiopathic Pulmonary Fibrosis Patients Using Quantitative High-Resolution Computed Tomography: A Retrospective Analysis

Author

Palmucci, Stefano, Torrisi, Sebastiano E., Falsaperla, Daniele, Stefano, Alessandro, Torcitto, Alfredo G., Russo, Giorgio, Pavone, Mauro, Vancheri, Ada, Mauro, Letizia A., Grassedonio, Emanuele, Sambataro, Gianluca, Puglisi, Silvia, Piciucchi, Sara, Tomassetti, Sara, Poletti, Venerino, Basile, Antonio, and Vancheri, Carlo

Published
2020
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7. Gastrointestinal pirfenidone adverse events in idiopathic pulmonary fibrosis depending on diet:the MADIET clinical trial

Author

Molina-Molina, Maria, Shull, Jessica Germaine, Vicens-Zygmunt, Vanesa, Rivera-Ortega, Pilar, Antoniou, Katerina, Bonella, Francesco, Renzoni, Elisabetta, Russell, Anne Marie, Maher, Toby M., Vancheri, Ada, Bachs, Anna, Avilés, Victoria, Palma, Josep, Bermudo, Guadalupe, Suarez-Cuartin, Guillermo, Tebé, Cristian, Rigo-Bonnin, Raul, Montes-Worboys, Ana, Wijsenbeek, Marlies, Vancheri, Carlo, Molina-Molina, Maria, Shull, Jessica Germaine, Vicens-Zygmunt, Vanesa, Rivera-Ortega, Pilar, Antoniou, Katerina, Bonella, Francesco, Renzoni, Elisabetta, Russell, Anne Marie, Maher, Toby M., Vancheri, Ada, Bachs, Anna, Avilés, Victoria, Palma, Josep, Bermudo, Guadalupe, Suarez-Cuartin, Guillermo, Tebé, Cristian, Rigo-Bonnin, Raul, Montes-Worboys, Ana, Wijsenbeek, Marlies, and Vancheri, Carlo

Published
2023

10. A New Method for the Assessment of Myalgia in Interstitial Lung Disease: Association with Positivity for Myositis-Specific and Myositis-Associated Antibodies

Author

Sambataro, Gianluca, primary, Ferrara, Chiara Alfia, additional, Spadaro, Carla, additional, Torrisi, Sebastiano Emanuele, additional, Vignigni, Giovanna, additional, Vancheri, Ada, additional, Muscato, Giuseppe, additional, Del Papa, Nicoletta, additional, Colaci, Michele, additional, Malatino, Lorenzo, additional, Palmucci, Stefano, additional, Cavagna, Lorenzo, additional, Zanframundo, Giovanni, additional, Ferro, Francesco, additional, Baldini, Chiara, additional, Sambataro, Domenico, additional, and Vancheri, Carlo, additional

Published
2022
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12. Differences between Acute Exacerbations of Idiopathic Pulmonary Fibrosis and Other Interstitial Lung Diseases

Author

Faverio, Paola, primary, Stainer, Anna, additional, Conti, Sara, additional, Madotto, Fabiana, additional, De Giacomi, Federica, additional, Della Zoppa, Matteo, additional, Vancheri, Ada, additional, Pellegrino, Maria Rosaria, additional, Tonelli, Roberto, additional, Cerri, Stefania, additional, Clini, Enrico M., additional, Mantovani, Lorenzo Giovanni, additional, Pesci, Alberto, additional, and Luppi, Fabrizio, additional

Published
2021
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13. Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease: where to go from here?

Author

Kalluri, Meena, primary, Luppi, Fabrizio, additional, Vancheri, Ada, additional, Vancheri, Carlo, additional, Balestro, Elisabetta, additional, Varone, Francesco, additional, Mogulkoc, Nesrin, additional, Cacopardo, Giulia, additional, Bargagli, Elena, additional, Renzoni, Elisabetta, additional, Torrisi, Sebastiano, additional, Calvello, Mariarosaria, additional, Libra, Alessandro, additional, Pavone, Mauro, additional, Bonella, Francesco, additional, Cottin, Vincent, additional, Valenzuela, Claudia, additional, Wijsenbeek, Marlies, additional, and Bendstrup, Elisabeth, additional

Published
2021
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14. "Usual" interstitial pneumonia with autoimmune features: a prospective study on a cohort of idiopathic pulmonary fibrosis patients

Author

Sambataro, Gianluca, primary, Ferrara, Chiara A., additional, Torrisi, Sebastiano E., additional, Spadaro, Carla, additional, Vignigni, Giovanna, additional, Vancheri, Ada, additional, Del Papa, Nicoletta, additional, Orlandi, Martina, additional, Colaci, Michele, additional, Malatino, Lorenzo, additional, Palmucci, Stefano, additional, Cavagna, Lorenzo, additional, Sambataro, Domenico, additional, and Vancheri, Carlo, additional

Published
2021
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15. Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease: where to go from here?

Author

Kalluri, M, Luppi, F, Vancheri, A, Vancheri, C, Balestro, E, Varone, F, Mogulkoc, N, Cacopardo, G, Bargagli, E, Renzoni, E, Torrisi, S, Calvello, M, Libra, A, Pavone, M, Bonella, F, Cottin, V, Valenzuela, C, Wijsenbeek, M, Bendstrup, E, Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elizabeth, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, Bendstrup, Elisabeth, Kalluri, M, Luppi, F, Vancheri, A, Vancheri, C, Balestro, E, Varone, F, Mogulkoc, N, Cacopardo, G, Bargagli, E, Renzoni, E, Torrisi, S, Calvello, M, Libra, A, Pavone, M, Bonella, F, Cottin, V, Valenzuela, C, Wijsenbeek, M, Bendstrup, E, Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elizabeth, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, and Bendstrup, Elisabeth

Abstract

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.

Published
2021

16. Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease:Where to go from here?

Author

Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elisabetta, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, Bendstrup, Elisabeth, Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elisabetta, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, and Bendstrup, Elisabeth

Abstract

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.

Published
2021

19. Does “UIPAF” really exist?

Author

Sambataro, Gianluca, primary, Libra, Alessandro, additional, Puglisi, Silvia, additional, Pavone, Mauro, additional, Pino, Fabio, additional, Torrisi, Sebastiano E., additional, Sardo, Giusy, additional, Vancheri, Ada, additional, Palmucci, Stefano, additional, Tomassetti, Sara, additional, Poletti, Venerino, additional, and Vancheri, Carlo, additional

Published
2020
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22. Cryptogenic Organizing Pneumonia: Evolution of Morphological Patterns Assessed by HRCT

Author

Tiralongo, Francesco, primary, Palermo, Monica, additional, Distefano, Giulio, additional, Vancheri, Ada, additional, Sambataro, Gianluca, additional, Torrisi, Sebastiano Emanuele, additional, Galioto, Federica, additional, Ferlito, Agata, additional, Fazio, Giulia, additional, Foti, Pietro Valerio, additional, Mauro, Letizia Antonella, additional, Vancheri, Carlo, additional, Palmucci, Stefano, additional, and Basile, Antonio, additional

Published
2020
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23. HRCT Patterns of Drug-Induced Interstitial Lung Diseases: A Review

Author

Distefano, Giulio, primary, Fanzone, Luigi, additional, Palermo, Monica, additional, Tiralongo, Francesco, additional, Cosentino, Salvatore, additional, Inì, Corrado, additional, Galioto, Federica, additional, Vancheri, Ada, additional, Torrisi, Sebastiano E., additional, Mauro, Letizia A., additional, Foti, Pietro V., additional, Vancheri, Carlo, additional, Palmucci, Stefano, additional, and Basile, Antonio, additional

Published
2020
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25. Disease progression across the spectrum of idiopathic pulmonary fibrosis: A multicentre study

Author

Sgalla, Giacomo, primary, Lo Greco, Erminia, additional, Calvello, Mariarosaria, additional, Varone, Francesco, additional, Iovene, Bruno, additional, Cerri, Stefania, additional, Donatelli, Pierluigi, additional, Vancheri, Ada, additional, Pavone, Mauro, additional, Luppi, Fabrizio, additional, Vancheri, Carlo, additional, and Richeldi, Luca, additional

Published
2020
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26. Quantitative assessment of interstitial lung disease in Sjögren’s syndrome

Author

Guisado-Vasco, Pablo, primary, Silva, Mario, additional, Duarte-Millán, Miguel Angel, additional, Sambataro, Gianluca, additional, Bertolazzi, Chiara, additional, Pavone, Mauro, additional, Martín-Garrido, Isabel, additional, Martín-Segarra, Oriol, additional, Luque-Pinilla, José Manuel, additional, Santilli, Daniele, additional, Sambataro, Domenico, additional, Torrisi, Sebastiano E., additional, Vancheri, Ada, additional, Gutiérrez, Marwin, additional, Mejia, Mayra, additional, Palmucci, Stefano, additional, Mozzani, Flavio, additional, Rojas-Serrano, Jorge, additional, Vanchieri, Carlo, additional, Sverzellati, Nicola, additional, and Ariani, Alarico, additional

Published
2019
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27. THU0563 COMPARISON BETWEEN PATIENTS WITH IDIOPATHIC PULMONARY FIBROSISAND INTERSTITIAL PNEUMONIA WITH AUTOIMMUNE FEATURES: A PROSPECTIVE COHORT

Author

Lorenzo Malatino, Mauro Pavone, Vancheri Ada, Sebastiano Emanuele Torrisi, Francesca Pignataro, Domenico Sambataro, Nicoletta Del Papa, Gianluca Sambataro, Michele Colaci, Stefano Palmucci, and Carlo Vanchieri

Subjects
medicine.medical_specialty, High-resolution computed tomography, medicine.diagnostic_test, business.industry, Interstitial lung disease, Retrospective cohort study, medicine.disease, Connective tissue disease, respiratory tract diseases, Pulmonary function testing, Idiopathic pulmonary fibrosis, Internal medicine, Cohort, medicine, business, Prospective cohort study
Abstract

Background: The term “Interstitial Pneumonia with Autoimmune Features (IPAF)” is used to describe patients with Interstitial Lung Disease in combination to clinical, serological and/or pulmonary features that are suggestive, but insufficient to satisfy classification criteria of a specific Connective Tissue Disease (CTD). Some retrospective studies available in literature described patient cohorts with IPAF heterogenous in terms of clinical, serological, and radiographic manifestations Objectives: To prospectively recruit a cohort of consecutive ILD patients classified as IPAF or as affected by Idiopathic Pulmonary Fibrosis (IPF); to describe their clinical, serological, and radiological features by a multi-disciplinar team composed by Pulmonologists, Radiologists, and Rheumatologists, comparing IPAF and IPF patients. Methods: In the lasts 2 years, they were enrolled 45 patients with IPAF and 143 with IPF among a total of 506 patients with Interstitial Lung Disease (ILD). All patients were evaluated clinically by both rheumatologists and pulmonologists, also by means of chest high resolution computed tomography (hrCT), pulmonary function tests (PFT), and nailfold videocapillaroscopy. Results: In IPAF cohort the most common characteristics from the clinical, serological and morphological domain were Raynaud’s phenomenon (RP) (31.1%) antinuclear antibodies positivity with titre ≥1/320 or any titre for centromeric or nucleolar pattern (17.7%) and nonspecific interstitial pneumonia (68.8%) respectively. The majority of patient (88.9%) had the minimum of 2 criteria at the recruitment. Female gender was more common in IPAF compared to IPF (62.2% vs 23%, p Conclusion: IPAF criteria recruit a rare cohort of patients with a disease probably less severe than IPF, even though clinically relevant. Further prospective studies may better define the long-term prognosis of ILD in IPAF. The follow-up of these patients by a multi-disciplinary team may be useful in order to early recognize and treat the new cases of CTDs. Some IPAF criteria probably are useless due to their high specificity for definite CTD and the classification need a revision in the light of the prospective experiences. Nevertheless, IPAF classification can recruit incomplete form or early onset of CTD that can allow a timely treatment in patients without a structured damage. References: [1] Sambataro G, Sambataro D, Torrisi SE, Vancheri A, Pavone M, Rosso R, Schisano M, Crimi C, Pignataro F, Fischer A, Del Papa N, Vancheri C. State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances. Eur Respir Rev2018:27(148) Disclosure of Interests: None declared

Published
2019

28. THU0600 QUANTITATIVE INDEXES TO ASSESS THE INTERSTITIAL LUNG DISEASE, AND ITS EXTENSION, IN SJÖGREN’S SYNDROME

Author

Stefano Palmucci, Mário J. Silva, Oriol Martín-Segarra, Carlo Vanchieri, José Manuel Luque-Pinilla, Jorge Rojas-Serrano, Vancheri Ada, Domenico Sambataro, F. Mozzani, Pablo Guisado-Vasco, Nicola Sverzellati, Daniele Santilli, Alarico Ariani, Isabel Martín-Garrido, Marwin Gutierrez, Sebastiano Emanuele Torrisi, Gianluca Sambataro, Miguel Angel Duarte-Millán, Chiara Bertolazzi, Mayra Mejía, and Mauro Pavone

Subjects
medicine.medical_specialty, Lung, business.industry, Interstitial lung disease, Retrospective cohort study, Gold standard (test), respiratory system, medicine.disease, respiratory tract diseases, Pulmonary function testing, body regions, medicine.anatomical_structure, Lung disease, Internal medicine, Diffuse disease, Medicine, Sjogren s, business
Abstract

Background Interstitial lung disease (ILD) is the most frequent pulmonary impairment in Sjogren’s syndrome (SS). The diagnosis is challenging, as there are not specific tests (i.e. autoantibodies or pulmonary function tests) or symptoms. Chest CT is the gold standard. Semiquantitative visual scores (SQCT) estimate ILD extent, though burdened by relevant intra-, inter-rater variability. Quantitative chest CT (QCT) is a promising method to assess ILD severity Objectives: to verify if QCT assessment could identify SS subjects affected by ILD and to identify those SS patients with an extensive lung disease. Methods: in this multi-center, cross-sectional, and retrospective study, subjects affected by SS and with a chest CT were enrolled. A blind and centralized SQCT assessment was carried out in order to calculate both Goh and Taouli scores. An operator-independent analysis of all CTs with an open-source software (i.e. Horos), provided the QCT indexes. Patients were clustered in cohorts according to ILD extent. Differences in QCT indexes distribution were analyzed using non-parametric tests. Results 102 consecutive SS patients were enrolled. ILD prevalence was 36% (36/102). There was a difference in QCT indexes’ distribution in SS-ILD versus SS without ILD (p 20% (according to Goh score) had QCT indexes statistically different from those with a limited ILD extension (p 0.7; the best QTC index was: tSkew AUC 0.87 (95CI 0.78-0.95), p Conclusion QCT indexes are a serious alternative to visual scorings in ILD related to autoimmune diseases. This innovative tool will open up a potential research area in SS as it appears able to select ILD patients with an extensive lung impairment and likewise a worse prognosis. We are confident that in the next future, QCT will have a pivotal role in the diagnosis, monitoring and treatment pathway of ILD associated to SS. References [1] Silva M, Milanese G, Seletti V, Ariani A, Sverzellati N. Pulmonary quantitative CT imaging in focal and diffuse disease: current research and clinical applications. Br J Radiol2018; 91: 20170644. Disclosure of Interests None declared

Published
2019

35. State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances

Author

Sambataro, Gianluca, primary, Sambataro, Domenico, additional, Torrisi, Sebastiano Emanuele, additional, Vancheri, Ada, additional, Pavone, Mauro, additional, Rosso, Roberta, additional, Schisano, Matteo, additional, Crimi, Claudia, additional, Pignataro, Francesca, additional, Fischer, Aryeh, additional, Del Papa, Nicoletta, additional, and Vancheri, Carlo, additional

Published
2018
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37. Immediate Effects of Sforzesco ® Bracing on Respiratory Function in Adolescents with Idiopathic Scoliosis.

Author

Di Maria, Fabrizio, Vescio, Andrea, Caldaci, Alessia, Vancheri, Ada, Di Maria, Chiara, Sapienza, Marco, Testa, Gianluca, and Pavone, Vito

Subjects
ADOLESCENT idiopathic scoliosis, LUNG volume, FRETTING corrosion, MULTIPLE comparisons (Statistics), LINEAR equations, LUNGS, ONE-way analysis of variance
Abstract

The thoraco-lumbar bracing is an effective management of adolescent idiopathic scoliosis (AIS). Studies have shown that brace wearing reduces lung volume. Whether or not the Sforzesco brace, frequently used in Italy, affects lung volume has not been investigated. We studied the immediate effect of Sforzesco bracing on lung volumes in 11 AIS patients (10 F, 1 M; aged 13.6 ± 1.6 yrs) mean Cobb angle 26 ± 4.49 degrees. Lung function variables and the perceived respiratory effort were recorded twice, before and 5 min after bracing. The one-way analysis of variance repeated measures, and multiple comparison tests, showed that means of unbraced variables were not significantly different from the corresponding means of predicted values, whereas means under brace were significantly lower (p < 0.05) compared to both predicted and baseline values of respiratory variables. In addition, a significant correlation (p < 0.0001) was found between unbraced and braced values, and linear regression equations were calculated. A significant but clinically unimportant increase in perceived effort was observed under the brace. In conclusion, data indicate that lung function is not impaired in moderate AIS and that wearing the Sforzesco brace causes an immediate, predictable reduction of lung volumes. Data also suggest that the respiratory discomfort during brace wearing could not be due to respiratory function defects. [ABSTRACT FROM AUTHOR]

Published
2021
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38. "Usual" interstitial pneumonia with autoimmune features: a prospective study on a cohort of idiopathic pulmonary fibrosis patients.

Author

Sambataro G, Ferrara CA, Torrisi SE, Spadaro C, Vignigni G, Vancheri A, Del Papa N, Orlandi M, Colaci M, Malatino L, Palmucci S, Cavagna L, Sambataro D, and Vancheri C

Subjects
Humans, Prospective Studies, Retrospective Studies, Tomography, X-Ray Computed, Autoimmune Diseases complications, Autoimmune Diseases diagnostic imaging, Idiopathic Pulmonary Fibrosis diagnosis, Idiopathic Pulmonary Fibrosis diagnostic imaging, Lung Diseases, Interstitial diagnostic imaging, Lung Diseases, Interstitial etiology
Abstract

Objectives: The classification interstitial pneumonia with autoimmune features (IPAF) includes patients with interstitial lung disease (ILD) associated with autoimmune characteristics insufficient to reach classification criteria for a specific autoimmune disease (SAD). These criteria are divided into three domains: clinical, serological and morphological. The latter domain does not include the usual interstitial pneumonia (UIP) pattern, which is deemed not to be significantly associated with SAD. Therefore, the enrolment of these patients is more difficult, requiring at least one item from both of the other domains. The objective of this study is to evaluate the rate of progression towards SAD of a cohort of UIP patients satisfying only one IPAF domain (we called this group "UIPAF") compared with classic idiopathic pulmonary fibrosis (IPF)., Methods: We prospectively enrolled IPF patients with radiologic and/or histologic UIP pattern, followed jointly by rheumatologists and pulmonologists from January 2017 to January 2021, with a minimum follow-up of 12 months., Results: We enrolled 190 IPF patients, 38 (20%) of whom were classified as UIPAF. IPF and UIPAF patients were similar for general characteristics, severity and prognosis, at presentation and at annual check-up. However, 28.9% of UIPAF patients progressed towards SAD, compared with 2% of IPF patients (χ2=30.4, p≤0.0001)., Conclusions: The association between a single clinical or serological domain of IPAF and UIP pattern is predictive for the development of a SAD if compared with isolated UIP. ILD can be the first manifestation of SAD, even with a UIP pattern, therefore, the morphological domain of IPAF criteria could be removed.

Published
2022
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39. Interstitial Lung Disease in patients with Polymyalgia Rheumatica: A case series.

Author

Sambataro G, Sambataro D, Pignataro F, Torrisi SE, Vancheri A, Pavone M, Palmucci S, Del Papa N, and Vancheri C

Abstract

Introduction: Severe morning stiffness with painful involvement of the girdles are often referred by patients with Interstitial Lung Disease (ILD), but the association between ILD and Polymyalgia Rheumatica (PMR) is rarely reported. The purpose of the work is to describe a series of patients classified as having PMR with ILD., Material and Methods: We retrospectively enrolled patients with a diagnosis of PMR referred to our center during the previous year for respiratory symptoms. Data concerning clinical and serological manifestations suggesting Connective Tissue Disease (CTD), High-Resolution Chest Tomography (HRCT), and Pulmonary Function Tests (PFTs) were systematically collected in order to verify the diagnosis., Results: Fifteen out of seventeen PMR patients had ILD. Ten patients had a confirmed diagnosis of PMR, while in five patients a CTD was discovered. Seven patients showed a severe restrictive pattern at PFTs requiring oxygen supplementation (five with PMR and two with CTD). In thirteen patients pulmonary symptoms started before or together with muscular symptoms. Regarding HRCT patterns, patients showed a Nonspecific Interstitial Pneumonia in nine cases, Usual Interstitial Pneumonia (UIP) and possible UIP in two and three cases, and a single case of Organizing Pneumonia and Combined Pulmonary Fibrosis and Emphysema Syndrome., Conclusions: Lung involvement should be evaluated in PMR patients, especially if asthenia is poorly responsive to low doses of steroids. In these cases, the diagnosis should be re-evaluated in depth, looking for a seronegative Rheumatoid Arthritis, a clinically amyopathic myositis or Interstitial Pneumonia with Autoimmune features.

Published
2018
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