UP-TO-DATE IN PULMONARY AND EXTRAPULMONARY SARCOIDOSIS

Sarcoidosis is a multisystemic immune disorder of unknown cause characterized by the formation of epithelioid granuloma in involved organs particularly the lung and the lymphatic system. Consistent recent advances have been made on the clinical, therapeutical and pathogenetic aspects. Clinical aspects: Various clinical phenotypes are better characterized and efforts to grade the severity could facilitate genetic studies and therapeutic trials. Pulmonary hypertension and some infrequent or atypical pulmonary manifestations as well as some rare extrapulmonary localizations have been thoroughly studied. Sarcoidosis-like granulomatosis due to identified causes constitute interesting models and have to be differentiated from sarcoidosis. Therapeutical aspects: Anti-TNF alpha drugs have been investigated and infliximab has been proved efficient even though some limits to its efficiency have to be taken into account. Pathogenetic aspects: Finally, advances concerning the pathogenesis, particularly genetic predisposing factors and phenotype/genotype correlations have been made.