Familial Pulmonary Fibrosis in Two Referral Centers in Northeast of Brazil.

<bold>Introdution:</bold> Familial Pulmonary Fibrosis (FPF) is defined as a case of fibrotic ILD with one or more relatives with a fibrotic form of interstitial lung disease (ILD). Although idiopathic pulmonary fibrosis(IPF) is the most common subtype, patients with FPF exhibit diferent phenotypes. Data on FPF from Brazil are scarce. <bold>Objective:</bold> To analyze clinical features and relative frequence of subtypes of ILD in FPF patients in Bahia, a state of northeast of country. <bold>Methodology:</bold> Clinical, functional and radiological data of a population(n = 35) with proven familial history were retrospectively collected. In 8 cases, lung tissue samples were obtained (surgical biopsies in 6 cases; transbronchial cryobiopsy in 2 cases). The diagnoses followed international guidelines and were reviewed by a multidisciplinary team. As this is an observational study of data collection only from medical records, we proposed waiving the informed consent form. The study was approved by the local research ethic committee. <bold>Results:</bold> Mean age was 66.7 ± 9.2, and 54% were female. Smoking history was reported by 39%, exposure to organic antigens has been observed in 58% (molds: 36% and avian antigens in 17%) and Innorganic antigens in 3% of cases. Autoantibodies were present at significant levels in 25% of patients and velcro crakles in 68%. Mean FVC was 69 ± 13%. Probable UIP was the most common radiologic pattern (33%), followed by UIP pattern (18%). The ILD subtypes observed were: Hypersensitivity Pneumonia (50%), IPF (16%), Unclassifiable (15%), CTD-ILD (10.5%) and CPFE (5.5%), others (3%). <bold>Conclusion:</bold> In our centers, HP was de most frequent ILD subtype in FPF group.