Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study

• DOI: 10.31138/mjr.31.3.330
• Publication Date: 2020-10-30T17:07:22Z
• Authors: Maria Karampeli; Konstantinos Thomas; Sofia Flouda; Aikaterina Chavatza; Dionisios Nikolop...; Antigone Pieta; Dimitrios Tseronis; Michail Aggelakos; Dimitra Kassara; Vasiliki Tzavara; Pelagia Katsimbri; Dimitrios Boumpas; Theofanis Karageo...
• Keywords: Original Paper; 03 medical and health sciences; 0302 clinical medicine; 3. Good health

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.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.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).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.