Notfälle beim sjögren-syndrom und der systemischen sklerose

Patients with primary Sjoegren's syndrome and systemic sclerosis commonly have a slowly progressive course of disease and often, emergencies are not assigned to the underlying rheumatic disease impeding timely diagnosis and treatment. Sjoegren's syndrome and Raynaud's syndrome are common presentations of a number of rheumatic diseases and may be accompanied by complications. In the present work, we describe typical cases of emergencies and provide background information including definition, pathogenesis, required diagnostics, and therapeutic options for complications. These include renal tubular acidosis, transverse myelitis, hyperviscosity syndrome in Sjoegren's syndrome, and pneumatosis, pulmonary artery hypertension, and renal crisis in systemic sclerosis. The work should help to diagnose these complications early and to allocate them to the underlying disease in order to avoid misinterpretation and to allow for timely and correct treatment.