Congophilic Fibrillary Glomerulonephritis: A Case Series
Congophilic Fibrillary Glomerulonephritis: A Case Series
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DOI：
10.1053/j.ajkd.2018.03.017
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发表时间：
2018-09-01
影响因子：
13.2
通讯作者：
Nasr, Samih H.
中科院分区：
文献类型：
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作者：
Alexander, Mariam P.;Dasari, Surendra;Nasr, Samih H.
理由与目的：偏振光下双折射刚果红阳性，传统上允许将有组织的肾小球沉积分为淀粉样或非淀粉样疾病。没有嗜血友病已被用来区分原纤维性肾小球肾炎（GN）和淀粉样变。我们描述了一系列刚果红阳性（嗜嗜性纤维性GN）沉积的纤维性GN病例，并讨论了DNAJB9在区分嗜嗜性纤维性GN和淀粉样变性中的作用。研究设计：病例系列。背景与对象：分析18例嗜血性原纤维性GN的临床病理特征。采用质谱法对24例刚果红阴性的原纤维GN、145例淀粉样变性和12例明显健康的个体进行比较。部分病例获得DNAJB9免疫组化。结果：在嗜嗜性纤维性GN病例中，质谱法未检测到淀粉样蛋白的蛋白质组学特征。DNAJB9是一种最近发现的纤维性GN的蛋白质组学标记物，在所有纤维性GN病例中，无论是否患有先天性亲血病，均使用质谱法检测到DNAJB9，在淀粉样变病例和健康个体中不存在。DNAJB9免疫组织化学证实了质谱分析结果。嗜血性原纤维性GN病例包括11名男性和7名女性，诊断时平均年龄为65岁。合并单克隆伽玛病、丙型肝炎病毒感染、恶性肿瘤或自身免疫性疾病分别在35%、22%、17%和11%的患者中存在。没有患者有肾外淀粉样变的证据。患者表现为蛋白尿（100%）、肾病综合征（47%）、血尿（78%）和慢性肾病（83%）。平均随访23个月后，31%的患者进展为终末期肾病，其余69%的患者肾功能持续下降。局限性：回顾性。未进行盲法病理评估。结论：有组织的原纤维沉积物的嗜嗜性不应仅仅依赖于鉴别原纤维GN和肾淀粉样变性。质谱法和DNAJB9免疫组织化学可用于进行这种区分。
Rationale & Objective: Congo Red positivity with birefringence under polarized light has traditionally permitted classification of organized glomerular deposits as from amyloid or nonamyloid diseases. The absence of congophilia has been used to differentiate fibrillary glomerulonephritis (GN) from amyloidosis. We describe a series of fibrillary GN cases in which the deposits are Congo Red-positive (congophilic fibrillary GN) and discuss the role of DNAJB9 in distinguishing congophilic fibrillary GN from amyloidosis.Study Design: Case series.Setting & Participants: Analysis of the clinicopathologic characteristics of 18 cases of congophilic fibrillary GN. Mass spectrometry was performed and compared with 24 cases of Congo Red-negative fibrillary GN, 145 cases of amyloidosis, and 12 apparently healthy individuals. DNAJB9 immunohistochemistry was obtained for a subset of cases.Results: The proteomic signature of amyloid was not detected using mass spectrometry among cases of congophilic fibrillary GN. DNAJB9, a recently discovered proteomic marker for fibrillary GN, was detected using mass spectrometry in all cases of fibrillary GN regardless of congophilia and was absent in cases of amyloidosis and in healthy individuals. DNAJB9 immunohistochemistry confirmed the mass spectrometry findings. The congophilic fibrillary GN cases included 11 men and 7 women with a mean age at diagnosis of 65 years. Concomitant monoclonal gammopathy, hepatitis C virus infection, malignancy, or autoimmune disease was present in 35%, 22%, 17%, and 11% of patients, respectively. No patient had evidence of extrarenal amyloidosis. Patients presented with proteinuria (100%), nephrotic syndrome (47%), hematuria (78%), and chronic kidney disease (83%). After a mean follow-up of 23 months, 31% of patients progressed to end-stage kidney disease and the remaining 69% had persistently reduced kidney function.Limitations: Retrospective nature. Blinded pathology evaluations were not performed.Conclusions: The congophilic properties of organized fibrillary deposits should not be solely relied on in differentiating fibrillary GN from renal amyloidosis. Mass spectrometry and DNAJB9 immunohistochemistry can be useful in making this distinction.