[Familial sarcoidosis in Poland: analysis of clinical characteristics and environmental aspects].
Sarcoidosis is a systemic disease of undetermined etiology although it can be related to genetic or environmental factors or both. We investigated 19 polish caucasian families with at least two affected relatives and healthy families members in order to evaluate the clinical aspects of familial sarcoidosis and the influence of environmental factors. We have found three types of familial sarcoidosis: 10 sib pairs, 1 sib triplet, 5 parent offspring pairs and one case of sarcoidosis in cousins. Most of sarcoidosis cases were histologically proved. A high frequency of chronic onset of the disease, chronic form with extrapulmonary sarcoidosis and fibrosis were observed. In only three families the course of the disease was similar in both affected relatives. There was no specific environmental agent found that could be related to the development of the disease. We concluded that it may be possible that familial sarcoidosis can have poorer prognosis that non-familial form, but there is no epidemiological data available about the clinical aspects of sarcoidosis in Poland. The clinical aspects of familial sarcoidosis can suggest the inherited susceptibility to the disease. The etiologic extrinsic factor has not been identified, but it doesn't exclude its role in the pathogenesis.
Goljan
A
A
Kliniki Chorób Płuc Instytutu Gruźlicy i Chorób Płuc, Warszawie.
Puścińska
E
E
Zych
D
D
Zieliński
J
J
D000328:Adult; D017677:Age Distribution; D017668:Age of Onset; D001706:Biopsy; D001999:Bronchoscopy; D018450:Disease Progression; D004784:Environmental Monitoring; D062665:Epidemiological Monitoring; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016273:Occupational Exposure / Q000009:adverse effects / Q000032:analysis; D009790:Occupations / Q000145:classification; D011044:Poland / Q000453:epidemiology; D011379:Prognosis; D011859:Radiography; D012507:Sarcoidosis / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology / Q000235:genetics*; D017678:Sex Distribution