Galactose-deficient IgA1 in skin and serum from patients with skin-limited and systemic IgA vasculitis

JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

卷:81

Article

Neufeld, Matthias^1,2  Molyneux, Karen³  Pappelbaum, Karin, I¹  Mayer-Hain, Sarah¹  von Hodenberg, Christina^1,2  Ehrchen, Jan^1,2  Barratt, Jonathan³  Suzuki, Yusuke⁴  Sunderkoetter, Cord^1,5 

[1] Univ Munster, Dept Translat Dermatoinfectiol, Munster, Germany

[2] Univ Munster, Dept Dermatol, Munster, Germany

[3] Univ Leicester, Dept Infect Immun & Inflammat, Leicester, Leics, England

[4] Juntendo Univ, Dept Nephrol, Fac Med, Tokyo, Japan

[5] Martin Luther Univ Halle Wittenberg, Univ Hosp Halle, Dept Dermatol & Venereol, Ernst Grube Str 20, D-06120 Halle, Saale, Germany

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

DOI  :  10.1016/j.jaad.2019.03.029

来源: Elsevier

【 摘 要 】

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. GDIgA1 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. Limitations: 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. Conclusion: 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 GDIgA1 in IgAV.

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