A patient presenting with isolated hematuria and renal dysfunction as rare manifestation of cryoglobulinemic glomerulonephritis in the course of autoimmune diseases including Sj?gren’s syndrome
A patient presenting with isolated hematuria and renal dysfunction as rare manifestation of cryoglobulinemic glomerulonephritis in the course of autoimmune diseases including Sj?gren’s syndrome
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一名患者出现孤立性血尿和肾功能障碍，这是包括干燥综合征在内的自身免疫性疾病过程中冷球蛋白血症性肾小球肾炎的罕见表现
DOI：
10.1007/s13730-018-0329-y
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发表时间：
2018
期刊：
影响因子：
1
通讯作者：
Uchida Shunya
中科院分区：
文献类型：
--
作者：
Yamanaka Masaki;Fujigaki Yoshihide;Kono Hajime;Nagura Michito;Arai Shigeyuki;Tamura Yoshifuru;Ota Tatsuru;Shibata Shigeru;Kondo Fukuo;Yamaguchi Yutaka;Uchida Shunya
自身免疫性疾病有时与免疫介导的肾脏疾病有关，冷球蛋白血症是原因之一。与原发性干燥综合征相关的冷球蛋白血症和冷球蛋白血症性肾小球肾炎是非丙型肝炎病毒相关疾病中最常见的疾病。其典型的肾脏表现是大量蛋白尿伴镜下血尿和肾功能不全。我们描述了一名 72 岁女性病例，她患有桥本氏病、自身免疫性肝炎、干燥综合征和免疫相关全血细胞减少症，并发冷球蛋白血症性肾小球肾炎。在进行肾活检之前，由于持续血尿但无明显蛋白尿，并且在 6 个月内出现轻度肾功能障碍，因此怀疑可能是由干燥综合征引起的肾小管间质性肾炎。与孤立性血尿相关的肾功能障碍在肾小球疾病中并不常见。然而，肾活检显示已确诊的膜增生性肾小球肾炎，伴有内皮下沉积物，由 IgM、IgG 和 C3 染色的管状结构组成。皮质类固醇加吗替麦考酚酯治疗成功地使肾功能正常化。医生不应忽视冷球蛋白血症性肾小球肾炎，即使尿液分析仅显示患有自身免疫性疾病的患者出现持续性孤立性血尿，这也可能导致预后不良。
Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren’s syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren’s syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis. Before kidney biopsy, tubulointerstitial nephritis probably due to Sjögren’s syndrome was suspected because of persistent hematuria without significant proteinuria and developing mild renal dysfunction over 6 months. The developing renal dysfunction associated with isolated hematuria is uncommon in glomerular diseases. Kidney biopsy, however, revealed established membranoproliferative glomerulonephritis with subendothelial deposits consisting of tubular structures with IgM, IgG, and C3 staining. Corticosteroids plus mycophenolate mofetil therapy successfully normalized renal function. Physician should not overlook cryoglobulinemic glomerulonephritis, which is potentially poor prognosis, even if urinalysis shows only persistent isolated hematuria in patients with autoimmune diseases.