Acute fibrinous and organizing pneumonia: A retrospective study

Abstract

Introduction: Acute Fibrinous and Organizing Pneumonia (AFOP) is a diffuse infiltrative pulmonary disease, characterized by presence of intra-alveolar fibrin and organizing pneumonia. It9s a rare and relatively unknown entity, with only a few cases described. Objectives: Evaluation and description of patients diagnosed with AFOP. Methods: Retrospective analysis of clinical characteristics, therapeutic approach and clinical course of patients diagnosed with AFOP. Results: We included 13 patients: mean age 54 years (SD 16), 7 (54%) female. Transthoracic needle aspiration biopsy guided by CT was performed in 8 (61%) patients, and surgical lung biopsy in 5 (39%). The mean time from onset of symptoms to diagnosis was 42 days (SD 34). The symptoms were: 9 (69%) fever, 5 (39%) cough, 4 (31%) chest pain, 3 (23%) dyspnea, 3 (23%) constitutional symptoms. The main radiological abnormality was bilateral ground glass/consolidation. The majority of patients (8) had hematologic malignancies background, 3 with autologous hematopoietic cell transplantation. In 7 patients more than one etiology could be identified: infections (6), drug induced toxicity (3), neoplasms (1). Two patients were considered to have idiopathic AFOP. Three (23%) patients had an acute presentation that required invasive mechanical ventilation. Regarding therapeutic approach, 10 (77%) patients were treated with corticosteroids, 3 (23%) mycophenolate mofetil, 2 (15%) cyclophosphamide. The median survival was 75 months, 6 (46%) patients are steel alive. Conclusions: AFOP had various etiologies and a significant clinical variety in clinical course and outcome in this cohort of patients. The survival seems to be related specially with underlying disease and etiology.