Zusammenfassung
Als nicht schubweise verlaufende Kollagenosen mit häufig langsamen und chronisch-progredienten Verlauf werden Notfälle häufig nicht der zugrunde liegenden Erkrankung zugeordnet, was ihre Diagnostik und schnelle Behandlung erschweren kann. Sjögren-Syndrom und Raynaud-Syndrom sind Begleitsymptom vieler rheumatischer Erkrankungen und können ebenfalls komplizierend verlaufen. In der folgenden Arbeit werden typische kasuistische Notfälle mit ihrer klinischen Symptomatik aufgeführt und anschließend ein Überblick über die pathophysiologische Zuordnung, die weitere Diagnostik, Differenzialdiagnostik und Therapie gegeben. Besonderes Augenmerk wird beim Sjögren-Syndrom auf Komplikationen der interstitiellen Nierenerkrankungen, auf die transverse Myelitis und das akute Hyperviskositätssyndrom gelegt. Bei der systemischen Sklerose wird auf die renale Krise oder auf Komplikationen der pulmonalen arteriellen Hypertonie eingegangen. Die Arbeit soll dazu dienen, Notfälle schneller zu erkennen und der zugrunde liegenden Erkrankung zuzuordnen, damit Überdiagnostik und Fehldiagnosen vermieden und Therapien frühzeitig eingeleitet werden.
Abstract
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.
Schlüsselwörter
Notfälle - Sjögren-Syndrom - systemische Sklerose - benigne Pneumatose - hypokaliämische Paralyse
Key words
emergencies - Sjoegren's syndrome - systemic sclerosis - pneumatosis cystoides - hypokalemic paralysis
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Korrespondenzadresse
PD Dr. G. Riemekasten
Charite
Immunology/Rheumatology
T170
10117 Berlin
Telefon: +49/30/450 61 30 17
Fax: +49/30/450 51 39 28
eMail: gabriela.riemekasten@charite.de
