Autoimmunity in Interstitial lung diseases: preliminary data from a tertiary center.

Abstract

Introduction: Interstitial lung diseases (ILD) involve lung interstitium and airspaces and can be either idiopathic or a complication of connective tissue diseases (CTD). Interstitial pneumonia with autoimmune features (IPAF) is a recently defined group of ILD showing some CTD features, but without fulfilling classification criteria for CTD. Aim of the study: to describe the main clinical, serological, instrumental and therapeutic features of a cohort of patients referred to our outpatient Immunology Clinic by pulmonologists, after multidisciplinary evaluation. Methods: clinical, laboratory and instrumental data were retrospectively collected from 60 patients. Results: Our cohort was divided into 3 groups: patients with a definite diagnosis of CTD (n=24); IPAF group (n=14); control group (n=22), including patients not fulfilling criteria for IPAF or a CTD, but with isolate presence of ILD. Main data are summarized in the attached figure. Conclusions: Despite the descriptive and retrospective design of our study, these data show that IPAF group exhibits worse lung function and 6MW tests performance, albeit their clinical and serological similarities with CTD. Our CTD group contains a high number of UIP, generally associated to a worse prognosis and linked to the high prevalence of rheumatoid arthritis. Surprisingly, their outcome is better than NSIP-related IPAF group.