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Interstitial lung disease in microscopic polyangiitis and granulomatosis with polyangiitis: demographic, clinical, serological and radiological features of an Italian cohort from the Italian Society for Rheumatology


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29
Collaborator/s: F. Schiavon1, E. Marasco2, A. Gattamelata3, R. Bortolotti4, D. Malandrino5, B. Maranini6

 

  1. Rheumatology Unit, Azienda Ospedaliera Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy. andreina.manfredi@gmail.com
  2. Rheumatology Unit, Azienda Ospedaliera Policlinico di Modena, University of Modena and Reggio Emilia, Modena, and Clinical and Experimental Medicine, PhD Program, University of Modena and Reggio Emilia, Modena, Italy.
  3. Rheumatology Unit, Department of Clinical Internal, Anaesthesiologic and Cardiovascular Sciences, Sapienza University of Rome, Italy.
  4. Department of Rheumatology, Hospital of Bruneck (ASAA-SABES), Italy.
  5. Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, and Rheumatology Unit, Santa Chiara Hospital, Trento, Italy.
  6. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital & Vita-Salute San Raffaele University, Milano, Italy.
  7. Rheumatology Unit, Santa Chiara Hospital, Trento, Italy.
  8. Rheumatology Section, Department of Medicine, University of Verona, and Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Italy.
  9. Division of Rheumatology, Prato Hospital, Prato, Italy.
  10. General Medicine Unit 1, Regional Centre for Rare Immunological and Rheumatological Diseases, Ca’ Foncello, University Hospital of Treviso, Italy.
  11. Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, University of Pavia, Italy.
  12. Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
  13. Rheumatology Unit, S. Anna Hospital (AZOU) Ferrara, Department of Medical Sciences, University of Ferrara, Italy.
  14. Rheumatology Clinic, Department of Medicine, University of Udine, ASU FC, Udine, Italy.
  15. Division of Rheumatology, ASST Gaetano Pini, Milan, Italy.
  16. Department of Surgical, Medical, Dental and Morphological Sciences related to Transplant, Oncology and Regenerative Medicine, Modena, Italy.
  17. Department of Experimental and Clinical Medicine, University of Firenze, Italy.
  18. Department of Rheumatology, Hospital of Bruneck (ASAA-SABES), Italy, and Department of Rheumatology, Medical University Graz, Austria.
  19. Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Italy.
  20. Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital & Vita-Salute San Raffaele University, Milano, Italy.
  21. Rheumatology Section, Department of Medicine, University of Verona, Italy.
  22. Division of Rheumatology, Prato Hospital, Prato, Italy.
  23. Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, University of Pavia, Italy.
  24. Rheumatology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena, Italy.
  25. Rheumatology Clinic, Department of Medicine, University of Udine, ASU FC, Udine, Italy.
  26. Division of Rheumatology, ASST Gaetano Pini, Milan, Italy.
  27. Rheumatology Unit, IRCCS Arcispedale Santa Maria Nuova, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy.
  28. Rheumatology Unit, Azienda Ospedaliera Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy.
  29. Rheumatology Unit, Azienda Ospedaliera Policlinico di Modena, University of Modena and Reggio Emilia, Modena, and Rheumatology Unit, IRCCS Arcispedale Santa Maria Nuova, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy.

  1. Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Italy.
  2. Department of Rheumatology, IRCCS Policlinico S. Matteo Fondazione, University of Pavia, Italy.
  3. Rheumatology Unit, Department of Clinical Internal, Anaesthesiological and Cardiovascular Sciences, Sapienza University of Rome, Italy.
  4. Rheumatology Unit, Santa Chiara Hospital, Trento, Italy.
  5. Department of Experimental and Clinical Medicine, University of Firenze, Italy.
  6. Rheumatology Unit, S. Anna Hospital (AZOU) Ferrara, Department of Medical Sciences, University of Ferrara, Italy.

Study Group of the Italian Society for Rheumatology (SIR)

CER15774
2023 Vol.41, N°4
PI 0821, PF 0828
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PMID: 36200955 [PubMed]

Received: 13/04/2022
Accepted : 25/07/2022
In Press: 06/10/2022
Published: 18/04/2023

Abstract

OBJECTIVES:
Interstitial lung disease (ILD) has been described as a possible pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), mainly granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Aim of this cross-sectional Italian national study was to describe demographic, clinical and serological profile of ILD related to MPA and GPA and investigate possible correlations between radiologic patterns of ILD and vasculitis features.
METHODS:
We enrolled 95 consecutive patients with AAV-ILD, 56 affected by MPA (58.9%) and 39 by GPA (41.1%).
RESULTS:
NSIP was the most frequently detected ILD pattern, observed in c-ANCA patients in 60.9% of cases, followed by UIP pattern mainly observed in p-ANCA patients (47.7%, p=0.03). ILD represented the first clinical manifestation, preceding vasculitis diagnosis in 22.1% of cases and, globally, ILD was already detectable at AAV diagnosis in 66.3% of patients. The diagnosis of ILD preceded that of AAV in 85.7% of p-ANCA positive-patients, while only one patient with c-ANCA developed ILD before AAV (p= 0.039). Multivariate analysis confirmed the correlation of UIP pattern with p-ANCA-positivity and a diagnosis of ILD before AAV, also when adjusted for age and sex.
CONCLUSIONS:
Our study confirms that UIP is a frequent pattern of lung disease in AAVILD patients. Our results also suggest that ILD can represent an early complication of AAV but also occur in the course of the disease, suggesting the need of a careful evaluation by both pulmonologist and rheumatologist to achieve an early diagnosis. Further prospective studies are needed to define ILD prevalence and evolution in AAV patients.

DOI: https://doi.org/10.55563/clinexprheumatol/xu4hmh

Rheumatology Article