Diagnostik und Therapie der Systemsklerose[*]

Zusammenfassung

Die Systemsklerose ist eine systemische Autoimmunerkrankung, die auch heute noch eine relativ ungünstige Prognose hat, vor allem, wenn eine pulmonale Beteiligung und/oder pulmonal-arterielle Hypertonie vorliegt. Bezüglich der Lungenfibrose ist erwiesenermaßen Cyclophosphamid effektiv, Anhaltspunkte für eine mögliche Wirksamkeit gibt es auch für Mycophenolat. ACE-Hemmer sind in der Lage, renale Krisen zu verhindern, und Endothelin-Antagonisten und Sildenafil können die pulmonale Hypertonie verbessern. Prostacyklinanaloga sind bei der Raynaud-Symptomatik wirksam, ebenso bei akralen Ulzera, letztere können auch mit Bosentan verhindert werden. Ansonsten ist die Datenlage leider sehr problematisch (wenig randomisierte Studien, oft mit sehr geringer Fallzahl). Die vorliegende Arbeit fasst die vorhandenen Studien zu den krankheitsmodifizierenden Therapieansätzen zusammen und gibt einen Überblick über die symptomatischen Therapieoptionen bei Raynaud-Symptomatik, Ulzera, Kalzifikationen, Arthritis und gastrointestinaler Problematik. Des Weiteren wird ein Überblick über die empfohlenen Verlaufskontrollen und diagnostischen Maßnahmen gegeben.

Abstract

Systemic sclerosis is a systemic autoimmune disease which still has a negative impact on overall survival. This especially holds true for the presence of pulmonary manifestations and/or pulmonary arterial hypertension. Cyclophosphamide is effective for pulmonary fibrosis, data also hint at an efficacy of mycophenolate mofetil. ACE inhibitors prevent renal crisis and endothelin antagonists and sildenafil improve pulmonary hypertension. Prostacyclin analogues are effective for the treatment of Raynaud`s phenomenon and digital ulcerations, which also can be prevented with bosentan. Otherwise, the data are sparse, there are few randomised studies with very small patient numbers. The present article summarises the studies on disease-modifying treatments and briefly reviews the symptomatic treatment options for Raynaud's phenomenon, ulcerations, calcifications, arthritis and gastrointestinal manifestations. Additionally, the recommended diagnostic measures and controls are reviewed.

Schlüsselwörter

Systemsklerose - Diagnostik - Therapie

Key words

systemic sclerosis - diagnostic measures - treatment

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5 *Die Arbeit entstand im Rahmen des Deutschen Netzwerkes Sklerodermie (DNSS).