A case of pathologically confirmed streptococcal infection-related IgA vasculitis with associated glomerulonephritis and leukocytoclastic cutaneous vasculitis
A case of pathologically confirmed streptococcal infection-related IgA vasculitis with associated glomerulonephritis and leukocytoclastic cutaneous vasculitis
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DOI：
10.1007/s13730-022-00684-4
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发表时间：
2022-02-14
期刊：
影响因子：
1
通讯作者：
Shimizu, Akira
中科院分区：
文献类型：
--
作者：
Inoue, Taichi;Takeuchi, Kazuhiro;Shimizu, Akira
我们报告一例80岁的妇女谁发展双侧下肢紫癜和肾功能损害与蛋白尿后几天短暂的发烧（第0天）。抗溶血素o抗体（ASO）和抗链激酶抗体（ASK）水平均较高，血清凝血因子XIII水平较低。皮肤活检显示白细胞破溃性血管炎，皮肤小血管中IgA和C3沉积，提示皮肤IgA血管炎。口服强的松龙后，皮肤病变有明显改善。肾功能和蛋白尿从第12天开始逐渐恶化。第29天进行肾活检，显示坏死性月牙状肾小球肾炎，肾小球系膜IgA和C3沉积。此外，半乳糖缺乏IgA1 （Gd-IgA1）沉积在肾小球和皮肤小血管中呈阳性，表明紫癜和肾小球肾炎具有相同的Gd-IgA1相关发病机制。此外，肾小球内肾炎相关纤溶酶受体（NAPlr）的沉积证实了急性链球菌感染与IgA血管炎之间的关联。除口服强的松龙治疗外，患者接受类固醇脉冲和静脉注射环磷酰胺治疗。肾功能和蛋白尿逐渐改善，但没有完全恢复，这是老年人IgA血管炎病程的典型表现。在本例中，肾小球内NAPlr和Gd-IgA1的沉积以及皮肤小血管内Gd-IgA1的沉积病理证实了链球菌感染相关的IgA血管炎。
We report the case of an 80 year-old woman who developed bilateral lower extremity purpura and renal impairment with proteinuria a few days after a transient fever (day 0). High levels of both anti-streptolysin-O antibody (ASO) and anti-streptokinase antibody (ASK), as well as low levels of coagulation factor XIII in serum were noted. Skin biopsy was performed and showed a leukocytoclastic vasculitis with deposition of IgA and C3 in the cutaneous small vessels, indicating IgA vasculitis in the skin. After initiation of oral prednisolone, the skin lesions showed significant improvement. However, renal function and proteinuria gradually worsened from day 12. Kidney biopsy was performed on day 29, which demonstrated a necrotizing and crescentic glomerulonephritis with mesangial deposition of IgA and C3. In addition, the deposition of galactose-deficient IgA1 (Gd-IgA1) was positive on glomeruli and cutaneous small vessels, indicating that the purpura and glomerulonephritis both shared the same Gd-IgA1-related pathogenesis. In addition, the association between the acute streptococcal infection and the IgA vasculitis was confirmed by the deposition of nephritis-associated plasmin receptor (NAPlr) in glomeruli. The patient was treated with steroid pulse and intravenous cyclophosphamide, in addition to the oral prednisolone treatment. Renal function and proteinuria gradually improved, but did not completely recover, as is typically seen with courses of IgA vasculitis in the elderly. In this case, the streptococcal infectionrelated IgA vasculitis was confirmed pathologically by the deposition of both NAPlr and Gd-IgA1 in glomeruli, as well as Gd-IgA1 in the cutaneous small vessels.