Sarcoidosis: recognition and treatment guidelines

Sarcoidosis is a systemic disorder of unknown aetiology characterised by
noncaseating granulomas leading principally to bilateral hilar lymphadenopathies,
pulmonary infiltration and skin and eye lesions. Sarcoidosis may involve other
organs, including peripheral lymph nodes, liver, spleen, nervous and
musculoskeletal systems, heart, ear, nose and kidney. Although the clinical
involvement of liver and heart is relatively uncommon, hepatic and cardiac
granulomas are present at autopsy in about 70 to 80% and 25 to 50%, respectively,
of patients with this disease. The diagnosis of sarcoidosis includes compatible
clinical and/or radiological presentations and histological evidence of
noninfectious 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 remissions, but it may become
progressive and chronic in a small percentage of patients. The optimal treatment
of sarcoidosis remains poorly defined. In patients with progressive pulmonary
dysfunction as well as in those with severe extrapulmonary localisations,
systemic corticosteroids usually represent the first approach, limited by long
term toxicity and frequent relapses after treatment interruption. In the presence
of refractory or corticosteroid-dependent forms of the disease, antimalarial
drugs or low dosage methotrexate may be used with prolonged benefit. The
indications for immunosuppressive agents such as azathioprine, chlorambucil,
cyclophosphamide and cyclosporin are uncommon and limited because of potentially
serious adverse effects and lack of information on their long term efficacy. In
the case of ocular and limited cutaneous manifestations, local corticosteroid
therapy may be useful.