晶体球蛋白诱发肾病的临床病理特征:病例系列。

IF 9.4 1区 医学 Q1 UROLOGY & NEPHROLOGY American Journal of Kidney Diseases Pub Date : 2024-06-21 DOI:10.1053/j.ajkd.2024.04.019
Samih H Nasr, Satoru Kudose, Anthony M Valeri, Ali Kashkouli, Samar M Said, Dominick Santoriello, Glen S Markowitz, Lihong Bu, Lynn D Cornell, Adel Samad, Jahangir Ahmed, Sanjeev Sethi, Nelson Leung, Vivette D D'Agati
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引用次数: 0

摘要

依据和目的:晶体球蛋白血症是一种以单克隆免疫球蛋白(MIgs)血管内结晶为特征的罕见综合征。有关肾脏受累的数据仅限于病例报告。本系列研究描述了晶体球蛋白诱发肾病(CIN)的临床病理学特征:病例系列:从梅奥诊所和哥伦比亚大学的肾病理学档案中发现了 19 例 CIN 病例。光镜(LM)和电子显微镜(EM)可见血管内(细胞外)MIg 晶体可定义为 CIN:在这些病例中,68%为男性,65%为白种人(中位年龄为 56 岁)。大多数患者表现为严重的急性肾功能衰竭(肌酐中位数为 3.5 毫克/分升)、血尿和轻度蛋白尿(中位数为 1.1 克)。常见的肾外表现有体质(67%)、皮肤(56%)和风湿(50%)。50%的病例有低补体血症。血液系统疾病包括肾脏单克隆丙种球蛋白病(MGRS)(72%)、淋巴瘤(17%)或骨髓瘤(11%),其中 65% 的疾病与 CIN 同时发现。所有患者都在 SPEP/SIF 中发现了 MIg(65% 为 IgGκ)。40%的患者sFLC比值超出肾脏范围,67%的患者通过骨髓活检发现了致病克隆。在 LM 中,晶体累及肾小球(100%)和血管(47%),通常伴有炎症反应(89%)和纤维蛋白(58%)。所有病例的电磁学检查均显示出晶体的亚结构(大部分为副结晶)。与冷冻组织相比,石蜡包埋组织的免疫荧光(IF)对晶体成分(63%为IgGκ)的显示更为敏感(92%对47%)。对 16 名患者进行了随访(中位数为 20 个月)。81%的患者接受了类固醇治疗,44%接受了血浆置换术,38%接受了血液透析,69%接受了化疗。90%接受克隆导向疗法的患者实现了肾功能恢复,而20%未接受克隆导向疗法的患者未实现肾功能恢复(P=0.017):局限性:回顾性设计,样本量小:CIN是一种罕见的与淋巴浆细胞性疾病(主要是MGRS)相关的肾病病因,通常表现为严重的AKI和肾外表现。诊断通常需要在石蜡包埋的肾组织上进行 IF。及时启动克隆导向疗法,同时使用皮质类固醇和血浆置换术,可使肾功能得到恢复。
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Clinicopathologic Characteristics of Crystalglobulin-Induced Nephropathy: A Case Series.

Rationale & objective: Crystalglobulinemia is a rare syndrome characterized by intravascular crystallization of monoclonal immunoglobulins (MIg). Data on kidney involvement are limited to case reports. This series characterizes the clinicopathologic spectrum of crystalglobulin-induced nephropathy (CIN).

Study design: Case series.

Setting & participants: Nineteen CIN cases identified from the nephropathology archives of Mayo Clinic and Columbia University. CIN was defined by intravascular (extracellular) MIg crystals visible by light microscopy (LM) and electron microscopy (EM).

Results: Among the cases, 68% were male, and 65% were Caucasian (median age, 56 years). Most patients presented with severe acute kidney injury (AKI) (median creatinine, 3.5mg/dL), hematuria, and mild proteinuria (median, 1.1g/day). Common extrarenal manifestations were constitutional (67%), cutaneous (56%), and rheumatologic (50%). Fifty percent of cases had hypocomplementemia. The hematologic disorders were monoclonal gammopathy of renal significance (MGRS) (72%), lymphoma (17%), or myeloma (11%), with 65% of these disorders discovered concomitantly with CIN. All patients had MIg identified on serum protein electrophoresis/immunofixation (IgGκ in 65%). The serum free light chain ratio was outside the renal range in 40%, and bone marrow biopsy detected the responsible clone in 67%. On LM, crystals involved glomeruli (100%) and vessels (47%), often with an inflammatory reaction (89%) and fibrin (58%). All cases exhibited crystal substructures (mostly paracrystalline) by EM. Immunofluorescence on paraffin-embedded tissue was more sensitive than frozen tissue (92% vs 47%) for demonstrating the crystal composition (IgGκ in 63%). Follow-up observation (median, 20 months) was available in 16 patients. Eighty-one percent received steroids, 44% plasmapheresis, 38% hemodialysis, and 69% chemotherapy. Ninety-percent of patients who received clone-directed therapy achieved kidney recovery versus 20% of those who did not (P=0.02).

Limitations: Retrospective design, small sample size.

Conclusions: CIN is a rare cause of nephropathy associated with lymphoplasmacytic disorders (mostly MGRS) and typically presents with severe AKI and extrarenal manifestations. Diagnosis often requires immunofluorescence performed on paraffin-embedded kidney tissue. Prompt initiation of clone-directed therapy, coupled with corticosteroids and plasmapheresis, may lead to recovery of kidney function.

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来源期刊
American Journal of Kidney Diseases
American Journal of Kidney Diseases 医学-泌尿学与肾脏学
CiteScore
20.40
自引率
2.30%
发文量
732
审稿时长
3-8 weeks
期刊介绍: The American Journal of Kidney Diseases (AJKD), the National Kidney Foundation's official journal, is globally recognized for its leadership in clinical nephrology content. Monthly, AJKD publishes original investigations on kidney diseases, hypertension, dialysis therapies, and kidney transplantation. Rigorous peer-review, statistical scrutiny, and a structured format characterize the publication process. Each issue includes case reports unveiling new diseases and potential therapeutic strategies.
期刊最新文献
Aiming for a Patient-Centered Organ Procurement and Transplantation Network. Exploring the Causal Relationship Between Kidney Function and Cancer Risk: Insights and Limitations of Mendelian Randomization. Proteinuria as an Endpoint in Clinical Trials of Focal Segmental Glomerulosclerosis. Sex, Acute Kidney Injury, and Age: A Prospective Cohort Study. Masthead
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