关键词: GD-IgA1 Henoch Schönlein purpura IgA nephropathy IgA vasculitis IgA vasculitis with nephritis IgAVN dermatology galactose-deficient IgA1

Mesh : Case-Control Studies Cohort Studies Female Humans Immunoglobulin A Male Middle Aged Skin / blood supply chemistry metabolism Skin Diseases, Vascular / blood metabolism Vasculitis / blood immunology metabolism

来  源:   DOI:10.1016/j.jaad.2019.03.029   PDF(Sci-hub)

Abstract:
BACKGROUND: IgA vasculitis (IgAV) encompasses a systemic form involving kidneys, gut, skin, or joints, and a skin-limited form. One characteristic feature of systemic IgAV is deposition of galactose-deficient IgA1 (GD-IgA1) in kidneys (as in IgA nephropathy). The relevance of GD-IgA1 for cutaneous vasculitis is unknown.
OBJECTIVE: We investigated whether GD-IgA1 is deposited perivascularly in systemic and also skin-limited IgAV and whether its serum levels differ between both forms.
METHODS: In a case-control study, deposition of GD-IgA1 was analyzed immunohistochemically by KM55 antibody in skin biopsy specimens from 12 patients with skin-limited IgAV and 4 with systemic IgAV. GD-IgA1 levels were compared by enzyme-linked immunosorbent assay in sera from 15 patients each with skin-limited and systemic IgAV and from 11 healthy individuals.
RESULTS: All biopsy samples from systemic IgAV, and also from skin-limited IgAV, revealed perivascular GD-IgA1 deposition. The average GD-IgA1 concentration in serum was significantly higher in systemic IgAV than in skin-limited IgAV, despite overlap between the groups.
CONCLUSIONS: Although high GD-IgA1 levels may be predictive of systemic IgAV, patient numbers were too low to determine cutoff values for systemic versus skin-limited IgAV.
CONCLUSIONS: Perivascular GD-IgA1 deposition is a prerequisite for systemic and skin-limited IgAV; however, high GD-IgA1 levels in some patients with systemic IgAV suggest a dose-dependent effect of GD-IgA1 in IgAV.
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