Zusammenfassung
Therapie und Verlauf der leukozytoklastischen Immunkomplexvaskulitis (LcV) hängen davon ab, ob IgA-haltige Immunkomplexe vorliegen [Purpura Schönlein-Henoch (PSH)] oder nicht. Eine LcV mit IgG- oder IgM-haltigen Immunkomplexen hat eine günstigere Prognose. Wenn kein Auslöser erkannt oder beseitigt werden kann, werden unkomplizierte Formen der LcV wegen des günstigen Spontanverlaufes symptomatisch behandelt. Wenn sich durch hämorrhagische Blasen Nekrosen ankündigen, sind vorübergehend Glukokortikoide angezeigt. Bei chronisch rezidivierender LcV bieten sich Dapson und Kolchizin an. Bei Erwachsenen mit PSH und schwerer Nierenbeteiligung gibt es keine ausreichenden Evidenzen für Besserung durch Glukokortikoide; zum Einsatz kommen je nach Symptomatik u. a. ACE-Hemmer. Bei der kryoglobulinämischen Vaskulitis werden zugrunde liegende Erkrankungen (Plasmozytom bzw. Hepatitis C) behandelt, bei ausbleibendem Erfolg hilft u. a. eine Plasmapherese. Gegen die urtikarielle Vaskulitis werden zunächst Dapson oder Kolchicin angewendet, bei schweren Formen Immunsuppressiva. Bei ANCA-assoziierten Systemvaskulitiden ist eine aggressive Induktionstherapie zur Erzielung einer Remission notwendig, meist mit Glukokortikoiden und Cyclophosphamid. Bei der klassischen Polyarteriitis nodosa verbessern Glukokortikoide die Prognose, bei der Polyarteriitis nodosa cutanea sind zunächst Colchicin oder Dapson angemessen. Bei der im Alter häufigeren Riesenzellarteriitis muss sofort mit einer Glukokortikoidbehandlung begonnen werden. Bei der Livedo-Vaskulopathie sind antithrombotische Maßnahmen wichtig mit niedrig molekularem Heparin oder Antagonisten von Vitamin K, zur Erhaltungstherapie auch Dipyridamol und Aspirin.
Abstract
Treatment and course of leukocytoclastic immune-complex vasculitis (LcV) depend on absence or presence of IgA in immune complexes [Henoch-Schoenlein-Purpura (PSH)]. LcV due to IgG- or IgM-containing immune complexes has a better prognosis. If triggers cannot be detected or avoided, symptomatic treatments are usually sufficient due to a usually favourable course. When hemorrhagic blisters suggest incipient skin necrosis corticosteroids are indicated. For chronic or relapsing LcV we suggest colchicine or dapsone. In adults with PSH and severe glomerulonephritis there is insufficient evidence for the efficacy of glucocorticoids; but e. g. ACE inhibitors can be helpful depending on symptoms. In cryoglobulinemic vasculitis underlying diseases (often plasmocytoma or hepatitis C) should be treated, sometimes supplemented by plasmapheresis. Dapsone or colchicine are usually started for urticarial vasculitis. ANCA-associated systemic vasculitis requires rapid and aggressive induction therapy, usually with glucocorticoids and cyclophosphamide. In classic polyarteriitis nodosa glucocorticoids improve prognosis, in polyarteriitis nodosa cutanea colchicine or dapsone are more appropriate. Giant cell arteriitis requires rapid therapy with glucocorticoids. For livedo vasculopathy antithrombotic measures are required with low molecular heparin or antagonists to vitamin K, for maintenance dipyridamol und aspirin.
Abbreviations
- ACR:
-
American College of Rheumatology
- ANCA:
-
Anti-Neutrophil-Cytoplasmic-Antibody
- CHCC:
-
Chapel Hill Consensus Conference
- HUV:
-
Hypokomplementämische urtikarielle Vaskulitis
- HUVS:
-
Syndrom der hypokomplementämischen urtikariellen Vaskulitis
- LcV:
-
Leukozytoklastische Vaskulitis
- NUV:
-
Normokomplementämische urtikarielle Vaskulitis
- PAN:
-
Polyarteriitis (Panarteriitis) nodosa
- PSH:
-
Purpura Schönlein-Henoch
- SLE:
-
Systemischer Lupus erythematodes
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Danksagung
Die Arbeit an diesem Artikel wurde unterstützt vom DLR/BMBF Fkz 01 GM 0310 (C.S.) Deutsches Netzwerk Systemische Sklerodermie und IZKF Münster D15 (C.S.).
Teile dieses Artikels sind gemeinsamen deutsch- oder englischsprachigen Artikeln entlehnt, deren Koautoren und Mitarbeitern ich ausdrücklich danken möchte: allen voran Frau Dr. G. Bonsmann aus unserer Klinik in Münster, ebenso Herrn Prof. Dr. T. Luger, dem Direktor unserer Klinik, Herrn Prof. Dr. Johannes Roth aus dem Institut für Immunologie und der Kinderklinik am UKM, außerdem Frau Dr. Anca Sindrilaru aus der Hautklinik Ulm, früher am Institut für Experimentelle Dermatologie in Münster.
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Sunderkötter, C., de Groot, K. Therapie von Vaskulitiden und Vaskulopathien. Hautarzt 59, 382–393 (2008). https://doi.org/10.1007/s00105-008-1530-2
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DOI: https://doi.org/10.1007/s00105-008-1530-2
Schlüsselwörter
- Leukozytoklastische Vaskulitis
- Purpura Schoenlein-Henoch
- ANCA-positive Systemvaskulitis
- Polyarteriitis nodosa
- Riesenzellarteriitis