Multicenter Registry of Interstitial Lung Diseases in Brazil

The relative frequency of different ILD varies in different countries; in Brazil is unknown. The aim of present study was to describe the frequency of ILD in Brazil. Methods: Patients from 5 centers evaluated during 2014-2017 were included. All patients were evaluated by pulmonologists, radiologists and pathologists involved with ILD. IPF was characterized according ATS/ERS/JRS/ALAT 2011 guideline. HP was characterized as definitive, probable and possible. Diagnosis of CTD, including IPAF, followed standard criteria. Familial interstitial pneumonia was the final diagnosis when ILD in first degree relatives was reported. Results: A total of 1,015 patients with ILD were enrolled. Mean age was 61 ± 15 yrs.; 53% were female. FVC was 69.2 ± 19.7% of predicted. Drugs with potential to cause ILD were reported by 12%, GERD symptoms by 42%, occupational exposure by 13% and to possible etiologies for HP by 53%. Autoantibodies were detected in significant levels in 31% (ANA 10.5%). Biopsies contributory to diagnosis were obtained by bronchoscopy in in 97 (9.6%), by surgical lung biopsy in 162 (16.0%) and from diverse sites in 42 (4.1%). Final diagnoses are shown in Figure. Conclusions: CTD-ILD are the most common cause for ILD in Brazil (21.7%), followed by HP (19%). IPF was diagnosed in 10% of cases, familial interstitial pneumonia in 8%, sarcoidosis in 7%. In 9% of diagnosis was undefined.