[Immunoglobulin A vasculitis (IgAV)].

Immunglobulin-A-Vaskulitis (IgAV).

Publisher: Springer Country of Publication: Germany NLM ID: 0414162 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1435-1250 (Electronic) Linking ISSN: 03401855 NLM ISO Abbreviation: Z Rheumatol Subsets: MEDLINE

Publication: 2006- : Heidelberg : Springer
Original Publication: Darmstadt, Steinkopff.

IgA Vasculitis*/diagnosis ; IgA Vasculitis*/drug therapy ; Glomerulonephritis, IGA*/diagnosis ; Glomerulonephritis, IGA*/drug therapy ; Polyarteritis Nodosa*/complications ; Giant Cell Arteritis*/drug therapy ; Granulomatosis with Polyangiitis*/complications; Adult ; Child ; Humans ; Immunoglobulin A/therapeutic use ; Skin ; Immunosuppressive Agents/therapeutic use

IgA vasculitis (IgAV) is an immune complex-mediated vasculitis characterized by IgA1-dominant immune deposits in small vessels. It is the most common systemic vasculitis in childhood with a mostly uncomplicated and self-limiting course. Adults are less affected but the course is frequently more complicated and more frequently accompanied by renal involvement. IgAV characteristically manifests itself on the skin with palpable purpura and in joints, the kidneys and the gastrointestinal tract. In cases of incomplete or atypical symptoms a differential diagnostic work-up is required. A number of triggers have been suggested, especially infections and drugs. Disease management is tailored to organ manifestations and the severity of the symptoms. For children, optimized supportive care and targeted symptom relief are usually sufficient. Management of renal and gastrointestinal manifestations follows recommendations for ANCA-associated vasculitis and IgA nephropathy. Treatment options include glucocorticoids and immunosuppressive agents with varying and mostly insufficient evidence.
(© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

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Keywords: Henoch-Schönlein purpura; IgAV-associated nephritis; Management; Palpable purpura; Small vessel vasculitis
Local Abstract: [Publisher, German] Die Immunglobulin-A-Vaskulitis (IgAV) ist eine immunkomplexvermittelte Vaskulitis, die sich durch Ig(Immunglobulin)A1-dominante Immunablagerungen in den kleinen Gefäßen auszeichnet. Sie ist die häufigste systemische Vaskulitis im Kindesalter mit meist unkompliziertem, selbstlimitierendem Verlauf. Erwachsene erkranken seltener, der Verlauf ist jedoch häufig komplizierter und häufiger mit einer Nierenbeteiligung verbunden. Die IgAV manifestiert sich klassischerweise an der Haut mit einer palpablen Purpura, den Gelenken, den Nieren und dem Gastrointestinaltrakt. Bei inkompletter oder untypischer Symptomatik ist eine Differenzialdiagnostik erforderlich. Diverse Trigger werden diskutiert, hierunter insbesondere Infektionen und Medikamente. Das Management orientiert sich an der Organmanifestation und der Schwere der Erkrankung. Insbesondere bei Kindern ist meist eine primär symptomorientierte und supportive Therapie ausreichend. Das Management der renalen und gastrotintestinalen Manifestation orientiert sich an Empfehlungen zu ANCA(antineutrophile zytoplasmatische Antikörper)-assoziierten Vaskulitiden und zur IgA-Nephropathie. Die Therapieoptionen umfassen Glukokortikoide und Immunsuppressiva mit unterschiedlicher und meist ungenügender Evidenz.

0 (Immunoglobulin A)
0 (Immunosuppressive Agents)

Date Created: 20230602 Date Completed: 20230912 Latest Revision: 20230922

20240829

PMC10236391

10.1007/s00393-023-01355-0

37266676