Sarkoidose

Abstract

Sarcoidosis is a multisystem disorder of unknown aetiology. The pathogenetic principle is a granulomatous inflammatory reaction, probably caused by an overwhelming cell-mediated immune response to an antigen. The disorder is characterised by an inflammatory infiltration by non-caseating granulomas developing in the affected organs. In most cases the lung is involved (> 90%) and a “classical” bilateral hilar lymphadenopathy develops and/or an increase of mostly reticulo-nodular opacities can be observed in chest X-ray. Basically sarcoidosis can affect each and every organ system, and up to 30% of patients show extrapulmonary involvement. Essential diagnostic procedures include chest X-ray, chest CT, bronchoscopy, pulmonary function testing and complementary determination of laboratory parameters. The clinical course is highly variable; however, in general the prognosis of sarcoidosis is good, and in most cases spontaneous recovery can be expected. This specifically applies to highly acute courses such as bilateral hilar lymphadenopathy syndrome (Lofgren's syndrome). However, at least 10% of untreated cases develop chronic progressive courses with relevant impairment of organ systems eventually resulting in premature death (e.g. pulmonary fibrosis). If any therapy is required at all, long-term administration of glucocorticoids is the basic element of treatment in sarcoidosis. In rare cases the administration of more potent immunosuppressive drugs such as methotrexate, azathioprine, or cyclophosphamide is required.