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Granulomatosis with polyangiitis associated with IgA nephropathy

机译：肉芽肿合并IgA肾病的多血管炎

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摘要

Granulomatosis with polyangiitis (GPA), previously referred to as Wegener’s granulomatosis, is a rare necrotizing granulomatous vasculitis, especially in children. GPA affects small- to medium-sized vessels, leading to involvement of multiple organs, including the upper and lower respiratory tracts and kidneys. Glomerular lesions associated with GPA typically present as crescentic glomerulonephritis with necrotizing lesions, with little or no staining for immunoglobulins and complement proteins. We report a unique pediatric case of GPA associated with IgA nephropathy, a representative immune-mediated glomerular disease. The initial renal biopsy specimen revealed fibrous sclerosis and mild mesangial proliferation without deposition of IgA. However, after clinical remission of GPA by treatment, the serum IgA level continued to be significantly higher than normal, and her paranasal sinusitis was poorly controlled. An acute upper respiratory infection resulted in worsened urinary findings without any systemic signs of GPA. The second renal biopsy specimen revealed deposition of IgA and C3 in the mesangium. The patient was treated with oral prednisolone alone, which led to complete remission of proteinuria within 1 month. IgA nephropathy is possibly associated with GPA during remission stage, and serum IgA level may be a valuable indicator to predict its association.
机译：肉芽肿性多血管炎（GPA），以前称为韦格纳肉芽肿病，是一种罕见的坏死性肉芽肿性血管炎，尤其是在儿童中。 GPA影响中小型血管，导致多个器官受累，包括上，下呼吸道和肾脏。与GPA相关的肾小球病变通常表现为新月型肾小球肾炎，并伴有坏死性病变，免疫球蛋白和补体蛋白几乎没有染色。我们报告了与IgA肾病（一种代表性的免疫介导的肾小球疾病）相关的GPA的独特儿科病例。最初的肾脏活检标本显示纤维性硬化和轻度系膜增生，未沉积IgA。然而，通过治疗使GPA临床缓解后，血清IgA水平继续显着高于正常水平，并且她的鼻旁鼻窦炎控制不佳。急性上呼吸道感染导致泌尿系统状况恶化，而没有任何GPA的全身征象。第二份肾活检标本显示IgA和C3沉积在系膜中。患者仅接受口服泼尼松龙治疗，导致蛋白尿在1个月内完全缓解。 IgA肾病可能在缓解阶段与GPA相关，血清IgA水平可能是预测其关联的有价值的指标。

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