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狼疮性肾炎的临床-组织形态学特征,达卡某中心的经验。

Clinico-histomorphologic Characteristics of Lupus Nephritis, Experience at a Center at Dhaka.

机构信息

Department of Histopathology, Armed Forces Institute of Pathology, Dhaka, Bangladesh.

Department of Nephrology, Combined Military Hospital, Dhaka, Bangladesh.

出版信息

Saudi J Kidney Dis Transpl. 2021 Nov-Dec;32(6):1754-1763. doi: 10.4103/1319-2442.352438.

DOI:10.4103/1319-2442.352438
PMID:35946290
Abstract

Lupus nephritis (LN) is one of the most serious systemic lupus erythematosus complications since it is the major predictor of poor prognosis. We present the clinico-histomorphologic characteristics of LN at a center in Dhaka. The study group comprised diagnosed cases of LN on core-needle renal biopsy specimens received in the Department of Histopathology, Armed Forces Institute of Pathology, Dhaka, from January 2018 to June 2019. Histological evaluation and direct immunofluorescence (DIF) study was carried out on each specimen. Each case was classified according to the International Society for Nephrology/Renal Pathology Society (ISN/RPS) Classification 2003 of LN. A total of 104 (9.20%) LN cases were included of total 1130 nonneoplastic renal biopsy specimens. The mean age was 28.29 ± 12.24 years, with a male:female ratio of 1:4.47. According to the ISN/RPS 2003 classification, most of the LN belong to class IV (42, 40.38%), followed by class III (27, 25.96%), class II (22, 21.15%), and so on. The mean age of class IV LN was 25.95 ± 10.15 years, with M:F = 1:4.25. The mean urinary total protein (UTP) was 4.62 ± 4.47 g/24 h and the mean serum creatinine was 1.87 ± 1.12 mg/dL. On histopathology, 22 (52.38%) had crescent formation, 27 (64.28%) had wire loop formation with subendothelial thrombi, and 30 (71.42%) had necrotizing lesion. Interstitial fibrosis and tubular atrophy (IFTA) was ≥25% in eight (19.04%) class IV LN. Among the subclasses, most common was IV-G (A/C) - 16 (38.09%), followed by IV-S (A/C) - nine (21.42%) and IV-S (A) and IV-G (A) each eight (19.04%). In four (9.52%) cases, class IV + V lesion was found. The mean age of class III LN was 26 ± 11.02 years, with a male:female of 1:26. The mean UTP was 3.45 ± 2.4 and serum creatinine was 1.71 ± 1.51. Among these, crescent was found in eight (29.63%) cases, wire loop and subendothelial thrombi in 11 (37.04%) cases, and necrotizing lesions in eight (29.63%) cases. IFTA was >25% in 14.81% of cases of class III LN. Among 27 class III LN, 17 (62.96%) were classified as III (A) and 10 (37.04%) as III (A/C). Two class III LN had an association with class V and two have presented with TMA. The mean age of class II LN was 29.95+ 12.26 years, with M:F = 1:2.66. UTP was 3.53 ± 2.43 g/24 h and serum creatinine was 1.52 ± 1.5 mg/dL for class II LN. Histologically, these cases had focal/diffuse mesangial proliferation with 25% IFTA in 9.09% of cases. One of the class II LN had associated amyloidosis. Among class V LN, the mean age was 45.12 + 13.64 years, with M:F = 1:3. The mean UTP was 4.06 ± 1.71 g/24 h and serum creatinine was 1.64 ± 0.94 mg/dL. Histologically, 37.5% had >25% IFTA. The mean age of class I lesion was 27.25 ± 17.42 years with equal number of males and females. The mean serum creatinine level was 0.65 ± 0.18 mg/dL and UTP was 2.71 ± 2.3 g/24 h for class I lesion. Only one class VI LN case was found which had IFTA 40% and presented with generalized edema. On DIF, among 100 cases, 86 were found with full-house immune deposits of different intensity; IgM was lacking in 10 cases and IgA was lacking in seven cases, while three cases lacked C3 deposits. The mean activity index was 7.10 and the mean chronicity index was 3.23 among 69 cases of proliferative LN. Among different histological classes of LN, the prevalence of class IV was more in this study which had the most severe form of clinical presentation, biochemical parameters (raised serum creatinine level), and histological findings (crescent formation and IFTA).

摘要

狼疮性肾炎 (LN) 是系统性红斑狼疮最严重的并发症之一,因为它是预后不良的主要预测因素。我们介绍了达卡一个中心的 LN 的临床-组织形态学特征。研究组包括在 2018 年 1 月至 2019 年 6 月期间,在达卡武装部队病理学院组织病理学系收到的核心针肾活检标本中诊断为 LN 的病例。对每个标本进行组织学评估和直接免疫荧光 (DIF) 研究。根据国际肾脏病学会/肾脏病理学会 (ISN/RPS) 2003 年的分类,对每个病例进行分类。共纳入 104 例(9.20%)LN 病例,占 1130 例非肿瘤性肾活检标本的 10.20%。平均年龄为 28.29 ± 12.24 岁,男女比例为 1:4.47。根据 ISN/RPS 2003 分类,大多数 LN 属于 IV 级(42 例,占 40.38%),其次是 III 级(27 例,占 25.96%)、II 级(22 例,占 21.15%)等。IV 级 LN 的平均年龄为 25.95 ± 10.15 岁,男女比例为 1:4.25。平均尿总蛋白(UTP)为 4.62 ± 4.47 g/24 h,平均血清肌酐为 1.87 ± 1.12 mg/dL。在组织病理学上,22 例(52.38%)有新月体形成,27 例(64.28%)有线状环形成伴内皮下血栓,30 例(71.42%)有坏死性病变。8 例(19.04%)IV 级 LN 的间质纤维化和肾小管萎缩(IFTA)≥25%。在亚类中,最常见的是 IV-G(A/C)-16 例(38.09%),其次是 IV-S(A/C)-9 例(21.42%)和 IV-S(A)和 IV-G(A)各 8 例(19.04%)。有 4 例(9.52%)发现 IV+V 病变。III 级 LN 的平均年龄为 26 ± 11.02 岁,男女比例为 1:26。平均 UTP 为 3.45 ± 2.4,血清肌酐为 1.71 ± 1.51。在这些患者中,8 例(29.63%)有新月体形成,11 例(37.04%)有线状环和内皮下血栓形成,8 例(29.63%)有坏死性病变。14.81%的 III 级 LN 的 IFTA >25%。在 27 例 III 级 LN 中,17 例(62.96%)为 III(A),10 例(37.04%)为 III(A/C)。有 2 例 III 级 LN 与 V 级有关,有 2 例伴有 TMA。II 级 LN 的平均年龄为 29.95+12.26 岁,男女比例为 1:2.66。UTP 为 3.53 ± 2.43 g/24 h,血清肌酐为 1.52 ± 1.5 mg/dL。组织学上,这些病例有局灶性/弥漫性系膜增生,9.09%的病例 IFTA 为 25%。1 例 II 级 LN 合并淀粉样变性。V 级 LN 的平均年龄为 45.12+13.64 岁,男女比例为 1:3。平均 UTP 为 4.06 ± 1.71 g/24 h,血清肌酐为 1.64 ± 0.94 mg/dL。组织学上,37.5%的病例 IFTA>25%。I 级病变的平均年龄为 27.25 ± 17.42 岁,男女比例相等。平均血清肌酐水平为 0.65 ± 0.18 mg/dL,UTP 为 2.71 ± 2.3 g/24 h。仅发现 1 例 VI 级 LN 病例,IFTA 为 40%,表现为全身性水肿。在 DIF 中,在 100 例病例中,86 例有不同强度的全室免疫沉积物;10 例缺乏 IgM,7 例缺乏 IgA,而 3 例缺乏 C3 沉积物。69 例增生性 LN 中有 71.00%的平均活动指数和 32.33%的平均慢性指数。在不同组织学类型的 LN 中,IV 级的患病率最高,其临床表现、生化参数(升高的血清肌酐水平)和组织学发现(新月体形成和 IFTA)最为严重。

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