SARCOIDOSIS - RECOGNITION AND TREATMENT GUIDELINES

Sarcoidosis is a systemic disorder of unknown aetiology characterised by noncaseating granulomas leading principally to bilateral hilar lymp hadenopathies, pulmonary infiltration and skin and eye lesions. Sarcoi dosis may involve other organs, including peripheral lymph nodes, live r, spleen, nervous and musculoskeletal systems, heart, ear, nose and k idney. Although the clinical involvement of liver and heart is relativ ely uncommon, hepatic and cardiac granulomas are present at autopsy in about 70 to 80% and 25 to 50%, respectively, of patients with this di sease. The diagnosis of sarcoidosis includes compatible clinical and/o r radiological presentations and histological evidence of noninfectiou s and noncaseating epitheloid cell granulomas in the absence of other identifiable agents responsible for such histological lesions. Disease course is variable and usually characterised by frequent I emissions, bur it may become progressive and chronic in a small percentage of pa tients. The optimal treatment of sarcoidosis remains poorly defined. I n patients with progressive pulmonary dysfunction as well as in those with severe extrapulmonary localisations, systemic corticosteroids usu ally; represent the first approach, limited by long term toxicity and frequent relapses after treatment interruption. In the presence of ref ractory or corticosertoid-dependent forms of the disease, antimalarial drugs or low dosage methotrexate may he used with prolonged benefit. The indications fur immunosuppressive agents such a; azathioprine, chl orambucil, cyclophosphamide and cyclosporin are uncommon and limited b ecause of potentially serious adverse effects and lack of information on their long term efficacy. In the case of ocular and limited cutaneo us manifestations, local corticosteroid therapy may be useful.