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Mediterr J Rheumatol 2020;31(3):330-6
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Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study
Maria Karampeli1, Konstantinos Thomas2, Sofia Flouda3, Aikaterina Chavatza3, Dionisios Nikolopoulos3, Antigone Pieta3, Dimitrios Tseronis3, Michail Aggelakos3, Dimitra Kassara3, Vasiliki Tzavara1, Pelagia Katsimbri3, Dimitrios Boumpas3, Theofanis Karageorgas3
Authors Information

1. Department of Internal Medicine, General Hospital Korgialenio-Benakio HRC, Athens, Greece

2. 4th Department of Internal Medicine, “Attikon” University Hospital of Athens, Greece

3. Rheumatology and Clinical Immunology Unit, 4th Department of Internal Medicine, “Attikon” University Hospital of Athens, Greece

Konstantinos Thomas, Dionisios Nikolopoulos, Antigone Pieta, Pelagia Katsimbri, Dimitrios Boumpas, Theofanis Karageorgas

Abstract
Objectives: Interstitial pneumonia with autoimmune features (IPAF) refers to patients with interstitial lung disease and autoimmune features not fulfilling the classification criteria for a specific connective tissue disease. We sought to study the characteristics, disease progression, response to treatment and complications of patients with IPAF in 1-year follow-up period. Methods: Clinical and laboratory findings, comorbidities, medications, pulmonary function tests (PFTs), chest HRCT and complications during the one-year follow-up period were documented for each of the 39 enrolled patients with IPAF. Results: The mean age at the time of IPAF diagnosis was 63.2 (±11) years, and 62% of patients were female. The most common clinical features were arthritis (82%) and rash (54%-not included in the IPAF criteria). Antinuclear antibodies (ANA) (59%) and non-specific interstitial pneumonia (NSIP-61.5%) were the most prevalent autoantibodies and radiological pattern respectively. PFTs at 12 months from baseline stabilized or improved in 79.5% of patients (p> 0.05). Infections were observed in 23.1% of patients during the first and in 12.8% during the second semester of follow-up. Two patients (5.1%) required hospitalization. All infections occurred in patients with non- usual interstitial pneumonia (UIP) pattern (p=0.02). Conclusions: Arthritis and rash are among the most common features in IPAF suggesting rash could be included into IPAF criteria. Almost 80% of patients had stable/improved PFTs at the end of follow-up. Infections occurred mainly in the first semester of treatment and in patients with non-UIP radiological pattern probably due to higher doses of corticosteroids used in these patients.

Article Submitted: 13 May 2020; Revised Form: 21 Jun 2020; Article Accepted: 1 Jul 2020; Available Online: 30 Sep 2020

https://doi.org/10.31138/mjr.31.3.330

This work is licensed under a Creative Commons Attribution 4.0 International License. 

©Karampeli M, Thomas K, Flouda S, Chavatza A, Nikolopoulos D, Pieta A, et al.

Keywords: Interstitial lung diseases, connective tissue diseases, interstitial pneumonia with autoimmune features, pulmonary fibrosis