Clinicopathologic Significance of Predominant Lambda Light Chain Deposition in IgA Nephropathy.
Clinicopathologic Significance of Predominant Lambda Light Chain Deposition in IgA Nephropathy.
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DOI：
10.1016/j.ekir.2022.08.003
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发表时间：
2022-11
影响因子：
6
通讯作者：
Nachman, Patrick H.
中科院分区：
文献类型：
--
作者：
Ravipati, Prasanth;Freese, Rebecca L.;Royal, Virginie;Bu, Lihong;Canetta, Pietro;Gipson, Debbie;Kallash, Mahmood;Kiryluk, Krzysztof;Nast, Cynthia;Reich, Heather N.;Rheault, Michelle N.;Saha, Manish;Nachman, Patrick H.
IgA肾病（IgAN）与其他肾小球疾病的不同之处在于常以lambda / kappa轻链沉积为主。使用治愈肾小球肾病（CureGN） IgAN队列，我们旨在确定主要的lambda链沉积是否与更差的临床结果或更活跃的疾病的组织病理学标志物相关。根据轻链染色的强度对患者进行分类。λ - kappa强度评分差值≥1+定义为λ显性（LD）组，kappa- λ共显性（KL）组的差异值< 1+。我们比较了每个类别患者的临床病程，从肾活检时间和加入CureGN的时间到缓解时间（蛋白尿< 0.3 g/g），估计肾小球滤过率（eGFR）降低50%，或进展到终末期肾病（ESKD）。我们还分析了两组间组织病理特征的差异。在440例患者中，我们发现两组在基线临床特征、缓解率、eGFR降低50%或进展为ESKD方面均无显著差异。LD组患者IgG染色≥1+的频率略高。CureGN病理学家对234例患者的活检结果进行了回顾性分析，结果显示LD组毛细血管内细胞增多的频率更高（51.1%比36.3%,P = 0.04），但其他组织病理特征无显著差异。在IgAN中，我们发现lambda优势与毛细血管内高细胞性增加之间存在关联，但与临床结果无关。
IgA nephropathy (IgAN) differs from other glomerular diseases by the frequently predominant lambda over kappa light chain deposition. Using the Cure Glomerulonephropathy (CureGN) IgAN cohort, we aimed to determine whether predominant lambda chain deposition is associated with worse clinical outcomes or histopathologic markers of more active disease. Patients were categorized based on the intensity of light chain staining. The lambda dominant (LD) group was defined by a difference in intensity score of lambda minus kappa ≥ 1+ and the kappa-lambda codominant (KL) group by a difference < 1+. We compared the clinical course of patients in each category from the time of kidney biopsy and time of enrollment into CureGN to the time of remission (proteinuria < 0.3 g/g), 50% reduction in estimated glomerular filtration rate (eGFR), or progression to end-stage kidney disease (ESKD). We also analyzed differences in histopathologic characteristics between the 2 groups. Among 440 patients, we found no significant differences between groups in baseline clinical characteristics nor in rates of remission, 50% reduction in eGFR, or progression to ESKD. Patients in the LD group had a modestly greater frequency of IgG staining ≥ 1+. The biopsy results of 234 patients reviewed by CureGN pathologists revealed a greater frequency of endocapillary hypercellularity (51.1% vs. 36.3%, P = 0.04) in the LD group, but no other significant difference in histopathologic features. In IgAN, we found an association between lambda predominance and increased endocapillary hypercellularity, but no association with clinical outcomes.
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影响因子：
6
作者：
Selewski DT;Ambruzs JM;Appel GB;Bomback AS;Matar RB;Cai Y;Cattran DC;Chishti AS;D'Agati VD;D'Alessandri-Silva CJ;Gbadegesin RA;Hogan JJ;Iragorri S;Jennette JC;Julian BA;Khalid M;Lafayette RA;Liapis H;Lugani F;Mansfield SA;Mason S;Nachman PH;Nast CC;Nester CM;Noone DG;Novak J;O'Shaughnessy MM;Reich HN;Rheault MN;Rizk DV;Saha MK;Sanghani NS;Sperati CJ;Sreedharan R;Srivastava T;Swiatecka-Urban A;Twombley K;Vasylyeva TL;Weaver DJ;Yin H;Zee J;Falk RJ;Gharavi AG;Gillespie BW;Gipson DS;Greenbaum LA;Holzman LB;Kretzler M;Robinson BM;Smoyer WE;Flessner M;Guay-Woodford LM;Kiryluk K;CureGN Consortium
通讯作者：
CureGN Consortium
影响因子：
3.5
作者：
FAURE, GC;TANG, JG;BENE, MC
通讯作者：
BENE, MC
影响因子：
19.6
作者：
Cattran, Daniel C.;Coppo, Rosanna;Zhang, Hong
通讯作者：
Zhang, Hong
影响因子：
39.2
作者：
Levey AS;Stevens LA;Schmid CH;Zhang YL;Castro AF 3rd;Feldman HI;Kusek JW;Eggers P;Van Lente F;Greene T;Coresh J;CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration)
通讯作者：
CKD-EPI (Chronic Kidney Disease Epidemiology Collaboration)
影响因子：
39
作者：
Barbour, Sean J.;Coppo, Rosanna;Trimarchi, H.
通讯作者：
Trimarchi, H.