[Immunoglobulin A vasculitis (IgAV)]. / Immunglobulin-A-Vaskulitis (IgAV).
Z Rheumatol
; 82(7): 587-598, 2023 Sep.
Article
em De
| MEDLINE
| ID: mdl-37266676
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Temas:
Geral
Base de dados:
MEDLINE
Assunto principal:
Poliarterite Nodosa
/
Vasculite por IgA
/
Arterite de Células Gigantes
/
Granulomatose com Poliangiite
/
Glomerulonefrite por IGA
Tipo de estudo:
Diagnostic_studies
/
Guideline
Limite:
Adult
/
Child
/
Humans
Idioma:
De
Revista:
Z Rheumatol
Ano de publicação:
2023
Tipo de documento:
Article