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Variants of the clinical course of familial pulmonary sarcoidosis

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.