Familial pulmonary fibrosis: a heterogeneous spectrum of presentations.

Ana Beatriz Hortense Marcel Koenigkam Dos Santos Danilo Tadeu Wada Alexandre Todorovic Fabro Mariana Silva Lima Silvia Carla Sousa Rodrigues Rodrigo do Tocantins Calado José Antonio Baddini-Martinez

Published in: Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia (2019)

Patients with FPF can present with a wide variety of clinical features. Most HRCT scans of these patients exhibit patterns not typical of usual interstitial pneumonia. The family history of fibrotic lung diseases should be investigated in all patients under suspicion, regardless of their age.

Keyphrases

end stage renal disease
ejection fraction
newly diagnosed
chronic kidney disease
prognostic factors
peritoneal dialysis
pulmonary fibrosis
magnetic resonance imaging
patient reported outcomes
mass spectrometry
early onset
patient reported
mechanical ventilation