Clinical, Radiological, and Pathological Features of Interstitial Pneumonia with Autoimmune Features

Objective: Ongoing debates about the adequacy of the interstitial pneumonia with autoimmune features (IPAF) criteria highlight the need for further research. The objective of this study was to evaluate pulmonary involvement patterns alongside respiratory function parameters, clinical, radiological, pathological, and laboratory features, treatment choices, and the relationships among these findings.Methods: Patients who met the 2015 European Respiratory Society and American Thoracic Society IPAF classification criteria were included. Initial high-resolution computed tomography (HRCT) scans were independently evaluated by 2 pulmonologists and 1 radiologist. Interstitial pneumonia with autoimmune features patients were grouped by gender, HRCT patterns, and presence of Raynaud’s phenomenon (RP), and their characteristics were compared.Results: Of the 78 patients included in the study, 55.1% were male. The most frequent clinical features were RP and inflammatory arthritis or polyarticular morning joint stiffness. The most common autoantibodies were antinuclear antibodies (85.9%) and anti-double-stranded DNA (21.8%). The most prevalent radiologic pattern was nonspecific interstitial pneumonia (92.3%). All patients met the serological and morphological domains, while 45 (57.7%) fulfilled the clinical domain. Smoking history, weight loss, and dry mouth were found to be independent factors different between groups (P < .005). Photosensitivity and the presence of a fibrotic nonspecific interstitial pneumonia (f-NSIP) pattern were identified as independent predictors of RP.Conclusion: The certain findings should be carefully investigated as independently associated factors for IPAF in interstitial lung disease patients with autoimmune features: smoking history and weight loss in men, dry mouth in women, and photosensitivity and f-NSIP pattern in patients with RP.