Zusammenfassung
Während das primäre Raynaud-Phänomen (RP) nur sehr selten zu Ulzera führt, findet man bei vielen Patienten mit sekundärem RP im Rahmen der systemischen Sklerodermie (SSc) Nekrosen, Ulzera oder gar Gangräne. Daher ist eine früh einsetzende und gut abgestufte Therapie des RP erforderlich. Die Therapie umfasst physikalische Maßnahmen, Vermeidung der Auslöser sowie stadiengerecht eingesetzte Medikamente. Auf der Grundlage publizierter Studien und eigener Erfahrungen ergibt sich folgendes Vorgehen: a) bei primärem und unkompliziertem sekundären RP Kalziumantagonisten in genügend hoher Dosierung, b) bei SSc-assoziiertem sekundären RP mit Hypertonie, Herz- oder Nierenbeteiligung Angiotensin neutralisierende Substanzen, c) bei zusätzlichem Vorliegen einer Depression Ausnutzen der vasodilatatorischen Wirkung der Serotoninwiederaufnahmehemmer, d) bei Vorliegen von Ulzera oder deutlichen trophischen Störungen i.v.-Gaben von Iloprost, e) bei therapierefraktären Fällen und Gefährdung eines Fingergliedes Sildenafil, f) zur Rezidivprohylaxe bei schwerem RP der Endothelinrezeptorantagonist Bosentan. Hierbei unterliegen c–f den Bestimmungen des Off-Label-Gebrauchs. Durch rechtzeitigen Einsatz therapeutischer Maßnahmen können bei sekundärem RP die Komplikationen wie Ulzera und Amputationen verringert werden. Außerdem verzögert bei Patienten mit SSc die rechtzeitige Therapie des RP möglicherweise auch die fibrotischen Prozesse.
Abstract
While primary Raynaud phenomenon (RP) only rarely leads to complications, secondary RP when associated with systemic sclerosis (SSc) frequently results in necrosis, ulcers or even gangrene. Therefore timely therapeutic intervention is required. Management of RP includes physical therapy, avoidance of triggers, and a variety of medications, depending on the disease severity. On the basis of published studies and of our own experience we suggest: a) for primary and uncomplicated secondary RP, calcium channel blockers in adequate doses; b) for SSc-associated secondary RP with hypertension, cardial or renal involvement, drugs which interfere with angiotensin; c) in case of concomitant depression, exploitation of the vasodilatory effects of serotonin reuptake inhibitors, d) in presence of ulcers or marked trophic alterations, intravenous administration of iloprost, e) for recalcitrant cases and endangered digits, sildenafil, f) for prevention of severe relapses, the endothelin-receptor antagonist bosentan. The drugs mentioned in c–f are not approved for RP and used off-label. In patients with secondary SSc-associated RP, timely therapy can reduce the percentage of complications such as ulcers or amputations. In addition, timely treatment of RP possibly retards ensuing fibrotic processes.
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Danksagung
Die Arbeit an diesem Beitrag wurde unterstützt vom DLR/BMBF Fkz 01 GM 0310 (C.S.) Deutsches Netzwerk Systemische Sklerodermie.
Wir danken Frau Jeannette Crout, Bibliothek der Hautklinik des Universitätsklinikums Münster, für ihre Unterstützung bei der Literatursuche und Herrn Konrad Walker, Klinik für Dermatologie und Allergologie des Universitätsklinikums Ulm.
Interessenkonflikt
Der korrespondierende Autor weist auf eine Verbindung mit folgender Firma/Firmen hin: G.R. und C.S. haben von der Firma Actelion ein Honorar für Vorträge und Teilnahme an einem Workshop erhalten. G.R. hat gegen Honorar Vorträge für die Firmen Schering und Abbott gehalten. Der Artikel ist dennoch unabhängig und produktneutral.
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Sunderkötter, C., Riemekasten, G. Raynaud-Phänomen in der Dermatologie . Hautarzt 57, 927–942 (2006). https://doi.org/10.1007/s00105-006-1218-4
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DOI: https://doi.org/10.1007/s00105-006-1218-4