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Progression and prognosis of interstitial pneumonia with autoimmune features: a longitudinal, prospective, multi-centre study


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19

 

  1. Regional Referral Centre for Rare Lung Disease, A.O.U. Policlinico G.Rodolico-San Marco, University of Catania, and Artroreuma srl, Outpatient Clinic of Rheumatology, Mascalucia, Catania, Italy. dottorsambataro@gmail.com
  2. Artroreuma srl, Outpatient Clinic of Rheumatology, Mascalucia, Catania, and Department of Clinical and Experimental Medicine, Internal Medicine Unit, Division of Rheumatology, Cannizzaro Hospital, University of Catania, Italy.
  3. Regional Referral Centre for Rare Lung Disease, A.O.U. Policlinico G.Rodolico-San Marco, University of Catania, Italy.
  4. Department of Internal Medicine and Therapeutics, U.O.S. Transplant Center, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy.
  5. Department of Internal Medicine and Therapeutics, U.O.S. Transplant Center, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy.
  6. Department of Clinical and Experimental Medicine, Internal Medicine Unit, Division of Rheumatology, Cannizzaro Hospital, University of Catania, Italy.
  7. Department of Clinical and Experimental Medicine, Internal Medicine Unit, Division of Rheumatology, Cannizzaro Hospital, University of Catania, Italy.
  8. U.O.C. Reumatologia, A.O. San Camillo-Forlanini, Roma, Italy.
  9. U.O.C. Reumatologia, A.O. San Camillo-Forlanini, Roma, Italy.
  10. U.O.C. Reumatologia, A.O. San Camillo-Forlanini, Roma, Italy.
  11. Respiratory Diseases Unit, San Gerardo Hospital, Monza University of Milano-Bicocca, Italy.
  12. Respiratory Diseases Unit, San Gerardo Hospital, Monza University of Milano-Bicocca, Italy.
  13. Respiratory Diseases Unit, San Gerardo Hospital, Monza University of Milano-Bicocca, Italy.
  14. Rheumatology Unit, Azienda Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy.
  15. Respiratory Disease Unit, Azienda Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy.
  16. Rheumatology Unit, Azienda Policlinico di Modena, University of Modena and Reggio Emilia, Modena, Italy.
  17. Department of Medical-Surgical Sciences and Advanced Technologies G.F. Ingrassia, Radiology I Unit, University Hospital Policlinico G. Rodolico-San Marco, Catania, Italy.
  18. Department of Internal Medicine and Therapeutics, Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy.
  19. Regional Referral Centre for Rare Lung Disease, A.O.U. Policlinico G. Rodolico-San Marco, University of Catania, Italy.

CER16028
2023 Vol.41, N°5
PI 1140, PF 1148
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PMID: 36189910 [PubMed]

Received: 08/07/2022
Accepted : 19/09/2022
In Press: 03/10/2022
Published: 03/05/2023

Abstract

OBJECTIVES:
To evaluate the rate of progression towards specific autoimmune diseases (SADs) of a prospective, multi-centre cohort of patients classifiable as interstitial pneumonia with autoimmune features (IPAF).
METHODS:
IPAF patients were enrolled based on specific research criteria, and jointly followed by rheumatologists and pulmonologists for at least one year with clinical check-ups, serological exams including autoimmunity, capillaroscopy and high-resolution computed tomography (HRCT). Diagnostic assessment was repeated at least once a year, or earlier when deemed useful.
RESULTS:
We enrolled 191 IPAF patients through 95 different combinations of IPAF criteria. Of these, 24.1% progressed towards SAD, mainly in connective tissue diseases but also in microscopic polyangiitis. The IPAF patients who progressed were younger than stable IPAF patients (63±10 years vs. 68±9 years, p=0.002) and had a longer follow-up (36.9±18.7 vs. 29.3±15.7 months, p=0.007), but similar severity. No parameters were associated with overall progression, but some parameters were associated with the development of specific diagnoses: Sjögren’s syndrome with positivity for SSA (p=0.007, χ2 7.4); idiopathic inflammatory myopathy with mechanic’s hands (p=<0.0001, χ2 12.6), organizing pneumonia pattern (p=0.01, χ2 6.1), positivity for anti-Pm/scl (p=0.04 χ2 4.1) and anti-MDA5 (p=0.04, χ2 4.2); systemic sclerosis with palmar telangiectasias (p=<0.0001 2 18.3), positivity for anti-Scl70 (p=<0.0001 χ2 12.5) and anti-PM/Scl (p=0.001 χ2 10.1).
CONCLUSIONS:
IPAF patients had a rate of progression towards SAD similar to that reported in previous studies on undifferentiated connective tissue diseases, thus including some patients in which lung involvement could represent the first or even the sole clinical manifestation of a SAD.

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

Rheumatology Article