[Fatal outcome of a multisystemic sarcoidosis in a 54-year-old patient]

Sarcoidosis is a systemic granulomatous disease of unknown etiology in which the immune system is overstimulated. The intrathoracic lymph glands and the lungs are the most common site of involvement but all other organs and systems can be affected. Although the prognosis is mainly favourable there is a small group of patients who are resistant to therapy and will eventually succumb to their disease. There is no causative treatment but corticosteroids are the most effective therapeutic agents for sarcoidosis. If there is resistance to corticosteroids an immunsuppressive combination treatment is available. The immunsuppressive drugs (Azathioprin, Methotrexat, Ciclosporin, Pentoxifyllin, Thaliodomid) and the clinical effectiveness of the combination treatment are still under investigation.

We describe a 54-year-old patient admitted to our hospital because of abnormal liver function tests. The cause was a sarcoidosis of the liver but we found also an involvement of the lung. The chest x-ray did not show any pathological findings, the lung function tests - VC and FEV1 - were normal but the diffusion capacity (DLCO) was reduced to 60 %. The course of the disease was characterized by a progressive deterioration of the lung function and of the general condition (loss of weight, fatigue, decrease in vitality). In the further course colon, spleen, bone marrow and kidneys were involved and hypercalcemia, pancytopenia and hyperspleenism occur. The treatment consisted in the application of corticosteroids. We started with 30 mg prednison daily and reduced it over a period of nine weeks to 7.5 mg. In the periods of deterioration we increased the doses up to 40 mg daily and achieved a temporary improvement of the general condition and the function tests. But eventually the fatal outcome could not be prevented not even after the removal of the massive enlarged spleen. The patient died four years after the diagnosis was established. The cause of death was an advanced hepatic encephalopathy as a result of extensive granulomatous involvement of the liver by a multisystemic sarcoidosis.

We conclude that a steroid resistant multisystemic sarcoidosis should be treated early in the course with a combination of two immunsuppressive agents. We recommend Azathioprin in addition to corticosteroids. We refer to the corresponding literature.