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Nonlupus Full House Nephropathy: A Systematic Review
Clinical Journal of the American Society of Nephrology ( IF 9.8 ) Pub Date : 2024-03-26 , DOI: 10.2215/cjn.0000000000000438
Martina Uzzo 1, 2 , Andreas Kronbichler 3 , Federico Alberici 4 , Ingeborg Bajema 2
Affiliation  

h comparable outcomes.The identification of three pathogenetic categories provides further clues for a shared clinical and diagnostic approach to the disease. Background The presence of a full house pattern at immunofluorescence on kidney biopsy in a patient without clinical and laboratory features of systemic lupus erythematosus (SLE) has led to the descriptive term nonlupus full house nephropathy. This systematic review and meta-analysis focus on nonlupus full house nephropathy nomenclature, clinical findings, and outcomes. Methods In a reiterative process, all identified terms for nonlupus full house nephropathy and other medical subject headings terms were searched in PubMed. Out of 344 results, 57 records published between 1982 and 2022 were included in the analysis. Clinical data of single patients from different reports were collected. Patients were classified into three pathogenetic categories, which were compared according to baseline characteristics, treatments, and outcomes. Results Out of the 57 records, 61% were case reports. Nonlupus full house nephropathy was addressed with 17 different names. We identified 148 patients: 75 (51%) were men; median age 35 (23–58) years. Serum creatinine and proteinuria at onset were 1.4 (0.8–2.5) mg/dl and 5.7 (2.7–8.8) g/d. About half of patients achieved complete response. A causative agent was identified in 51 patients (44%), mainly infectious (41%). Secondary nonlupus full house nephropathy was mostly nonrelapsing with worse kidney function at onset compared with idiopathic disease (P = 0.001). Among the 57 patients (50%) with idiopathic nonlupus full house nephropathy, complete response was comparable between patients treated with immunosuppression and supportive therapy; however, proteinuria and creatinine at onset were higher in patients treated with immunosuppression (P = 0.09 and P = 0.07). The remaining 7 patients (6%) developed SLE after a median follow-up of 5.0 (1.9–9.0) years. Conclusions Our data support that SLE and nonlupus full house nephropathy are distinct clinical entities, with comparable outcomes. A small subset of patients develops SLE during follow-up. Nonlupus full house nephropathy is addressed by many different names in the literature. The identification of three pathogenetic categories provides further clues for the management of the disease....

中文翻译:

非狼疮性满屋肾病:系统评价

h 可比较的结果。三种致病类别的识别为该疾病的共同临床和诊断方法提供了进一步的线索。背景 对于没有系统性红斑狼疮 (SLE) 临床和实验室特征的患者,肾活检免疫荧光检查中出现满屋模式,导致了描述性术语非狼疮满屋肾病。本系统综述和荟萃分析重点关注非狼疮性全屋肾病的命名、临床表现和结果。方法 在重复过程中,在 PubMed 中检索所有已识别的非狼疮性满堂肾病术语和其他医学主题词术语。在 344 项结果中,分析包含 1982 年至 2022 年间发布的 57 项记录。收集来自不同报告的单个患者的临床数据。患者被分为三个发病类别,根据基线特征、治疗和结果进行比较。结果 57 条记录中,61% 为病例报告。非狼疮性满屋肾病有 17 个不同的名称。我们确定了 148 名患者:75 名(51%)是男性;中位年龄 35 (23–58) 岁。发病时血清肌酐和蛋白尿分别为 1.4 (0.8–2.5) mg/dl 和 5.7 (2.7–8.8) g/d。大约一半的患者达到完全缓解。在 51 名患者 (44%) 中确定了病原体,主要是传染性的 (41%)。与特发性疾病相比,继发性非狼疮性满屋肾病大多是非复发性的,且发病时肾功能较差(P = 0.001)。在 57 名特发性非狼疮性满屋肾病患者 (50%) 中,接受免疫抑制治疗和支持治疗的患者的完全缓解程度相当;然而,接受免疫抑制治疗的患者发病时蛋白尿和肌酐较高(P = 0.09 和 P = 0.07)。其余 7 名患者 (6%) 在中位随访 5.0 (1.9–9.0) 年后出现 SLE。结论 我们的数据支持 SLE 和非狼疮性满屋肾病是不同的临床实体,具有可比的结果。一小部分患者在随访期间出现系统性红斑狼疮。非狼疮性满屋肾病在文献中有许多不同的名称。三种致病类别的鉴定为疾病的管理提供了进一步的线索......
更新日期:2024-03-26
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