Introduction: Interstitial Lung Disease (ILD) patients (pts) with insufficient characteristics to reach criteria for Specific Autoimmune Disease (SAD) are classified as Interstitial Pneumonia with Autoimmune Features (IPAF). These criteria do not include Usual Interstitial Pneumonia (UIP) pattern in the morphological domain. We recently demonstrated a similar rate of progression to SAD between IPAF and a cohort of UIP-ILD in which only one of the other two domains (clinical/serological) was satisfied. We named this group “UIPAF” (Sambataro G. et al, Chest 2020). Aim: to analyze the clinical presentation and evolution of a cohort of UIPAF compared to Idiopathic Pulmonary Fibrosis (IPF). Methods: our ILD database was retrospectively reviewed for UIPAF and IPF pts. All pts between Jan-2017 and Jan-2019 were evaluated by rheumatologists and pulmonologists. A follow-up time of 1-year was required. Pts with SAD or IPAF were excluded. Results: 22 UIPAF and 78 IPF pts were studied. No differences were found in lung function, progression and mortality (table). Five UIPAF pts developed SAD within 1-year:2 Rheumatoid Arthritis, 1 Granulomatosis with Polyangiitis, 1 Polymyositis and 1 Systemic Sclerosis; no one within IPF pts (p=0.0001). Conclusions: UIPAF and IPF pts look indistinguishable for clinical presentation and outcome even if UIPAF have a higher probability to become a SAD. Progression of UIPAF is similar to “classic” IPAF, therefore the presence of a “morphological domain” in IPAF criteria need to be discussed.
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