Abstract
The aim of the study was to identify variants of the clinical course of FPS.
Methods: We found FPS in 26 patients from 1050 observed patients with pulmonary sarcoidosis (PS) for last 15 years. The group consisted of 12 families with 2 or more members suffering from PS. The mean age was 39±5,3 years. FPS was diagnosed after a clinical, radiological, functional and pathological evaluation in all 26 patients with FPS.
Results: In all members of 6 families the course of PS was favorable - spontaneous regression in 4 cases and under the influence corticosteroid therapy in 2 cases. The course of PS in 3 families characterized frequent relapses with extrapulmonary lesions. In members of 3 families we found advanced pulmonary sarcoidosis (APS). APS was characterized by progressive dyspnea, significant fibrosing and fibrocystic pulmonary lesions at CT (HRCT) lung scans, and a restrictive impairment with reduced gas exchange on pulmonary function test results (varying degrees of severity). Patients of this families received systemic corticosteroids in combination with cytotoxic agents, 2 patients died within the 15 and 24 years of illness. The cause of death was respiratory failure. We observed cancer in 2 cases and lymphoma in one case in FPS patients (after diagnostics of sarcoidosis).
Conclusion: Genetic predisposition plays a role in pulmonary sarcoidosis. FPS in half of cases had progressive and relapsing course and needs careful monitoring for evalution of dynamic of clinical symptoms, functional indicators, and radiological examination.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA1395.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019