Prognostic impact and clinical characteristics of Interstitial Pneumonia with Autoimmune features (IPAF) in a multidisciplinary setting

Abstract

Background: Clinical evolution and optimal management of IPAF are still undefined and need prospective assessment. Objectives: to define the characteristics and evolution of IPAF patients in a multidisciplinary setting. Methods: IPAF pts referring to multidisciplinary Rheumatology/Pneumology outpatient clinic. We excluded patients with diagnosis of Anti-synthetase Sd. Data were retrospectively collected. Results: we analyzed the first 25 pts (19 F, 76%, 6 M, 24%), with a median onset age of 67 years (IQR, 59-74) and follow-up of 32 months (IQR 22-69). ANA test was positive in 23 (92%) cases (cytoplasmic+ in 17:68%). Anti-ENA screen was + in 9: 36% pts. One pt was + for anti-PM-Scl, one for for anti-Mi2 and one for anti-Ku Ab. Pts had mainly a NSIP pattern (19, 76%, cases; with concomitant OP, in 3). Three patients had UIP-like and 3 OP pattern. The majority of patients (15, 60%) satysfied only the morphological and serological domains. Clinical domains satisfied were: arthritis (4) Raynaud’s ph. (5) palmar teleangectasias (2) mechanic’s hands (1) and Hikers feet (1). Clinical spectrum time course was variable in 5 pts: 3 pts developed arthritis after ILD, and 2 developed ILD respectively after arthritis and inflammatory miopathy. Three pts were admitted in ICU for Rapidly Progressive (RP) ILD and 2 died. All pts received low dose steroids in combination mainly with Azathioprine, Mycophenolate, Cyclosporin, Hydroxychloroquine. 92% of patients were alive at FU. Conclusions: A multidisciplinary approach is useful in the diagnosis and the management of IPAF. RP-ILD was common, prognosis of IPAF was highly variable.