[Non-tuberculous systemic granulomatosis mimicking sarcoidosis but related to a specific etiology. Study of 67 cases].

PURPOSE

Systemic granulomatosis (SG) are frequently encountered in internal medicine. Despite a large list of aetiologies, the investigations remain often negative leading to the diagnosis of atypical sarcoidosis. The spectrum of the causes, as well as evolution of these SG is not clearly delineated in the literature.

METHOD

We analyzed the case reports of all but tuberculous GS submitted at the National Meetings of the National French Society of Internal Medicine from 1990 to 2006.

RESULTS

Sixty-seven cases were included in the study. The average age at the beginning of the symptoms was 47.8 years and 28.4% of the patients were female. The median diagnostic delay was one year. General symptoms were present in 73.1% of the cases. The involved organs were the liver (46.3%), lungs (25.4%), lymph nodes (22.4%), digestive tract (16.4%), skin (16.4%), spleen (14.9%). The granuloma were detected mainly in the liver (38.8%), lymph nodes (17.9%), bone marrow (16.4%) and lungs (11.9%). Elevated erythrocyte sedimentation rate or increased C reactive protein serum levels were noted in 65.6% of the patients. Before diagnosis, 19.4% of the patients received a corticotherapy. The most common diagnoses were infections (65.6%) followed by drugs (19.5%), "toxic substances" or various foreign bodies (5.9%), neoplasias (5.9%) and immune deficiencies (3%). The evolution was favourable in 80% of the cases but 8.3% of the patients died. The disease course of the patients having received a corticotherapy prior to the diagnosis was more unfavourable with a death rate of 45%.

CONCLUSION

In atypical sarcoidosis (fever, advanced age, increased acute phase reactants...) a specific aetiology and especially an infectious disease should be ruled out before considering the diagnosis of sarcoidosis. Corticotherapy is a factor of poor prognosis.