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努力制定青少年增殖性狼疮性肾炎的达标治疗方案——德国和奥地利儿科风湿病学家和肾病学家的一项调查

Working Towards a Treat-to-Target Protocol in Juvenile Proliferative Lupus Nephritis - A Survey of Pediatric Rheumatologists and Nephrologists in Germany and Austria.

作者信息

Vollbach Kristina, Schuetz Catharina, Hedrich Christian M, Speth Fabian, Mönkemöller Kirsten, Brunner Jürgen, Neudorf Ulrich, Rietschel Christoph, Hospach Anton, Kallinich Tilmann, Hinze Claas, Wagner Norbert, Tönshoff Burkhard, Weber Lutz T, Latta Kay, Thumfart Julia, Bald Martin, Wiemann Dagobert, Zappel Hildegard, Tenbrock Klaus, Haffner Dieter

机构信息

Department of Pediatrics, RWTH Aachen University Hospital, Aachen, Germany.

Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.

出版信息

Front Pediatr. 2022 Apr 22;10:851998. doi: 10.3389/fped.2022.851998. eCollection 2022.

Abstract

BACKGROUND

To describe treatment practices for juvenile proliferative lupus nephritis (LN) class III and IV of pediatric rheumatologists and nephrologists in Germany and Austria in preparation for a treat-to-target treatment protocol in LN.

METHODS

Survey study by members of the Society for Pediatric and Adolescent Rheumatology (GKJR) and the German Society for Pediatric Nephrology (GPN) on diagnostics and (concomitant) therapy of LN.

RESULTS

Fifty-eight physicians completed the survey. Overall, there was a considerable heterogeneity regarding the suggested diagnostics and management of juvenile proliferative LN. Increased urinary protein excretion, either assessed by 24 h urine collection or spot urine (protein-creatinine ratio), and reduced estimated glomerular filtration rate were specified as important parameters for indication of kidney biopsy to diagnose proliferative LN and monitoring of therapy. Corticosteroids were generally proposed for induction and maintenance therapy, most often in conjunction with either mycophenolate mofetil (MMF) or cyclophosphamide (CP) as steroid-sparing immunosuppressants. MMF was clearly preferred over CP for induction therapy of LN class III, whereas CP and MMF were equally proposed for LN class IV. MMF was most often recommended for maintenance therapy in conjunction with oral corticosteroids and continued for at least 3 years and 1 year, respectively, after remission. Hydroxychloroquine was widely accepted as a concomitant measure followed by renin-angiotensin system inhibitors in cases of arterial hypertension and/or proteinuria.

CONCLUSION

The majority of pediatric rheumatologists and nephrologists in Germany and Austria propose the use of corticosteroids, most often in combination with either MMF or CP, for treatment of proliferative LN in children. The considerable heterogeneity of responses supports the need for a treat-to-target protocol for juvenile proliferative LN between pediatric rheumatologists and nephrologists.

摘要

背景

为制定狼疮性肾炎(LN)的靶向治疗方案做准备,描述德国和奥地利儿科风湿病学家和肾病学家对青少年增殖性狼疮性肾炎(III级和IV级)的治疗方法。

方法

由儿童和青少年风湿病学会(GKJR)及德国儿科肾脏病学会(GPN)成员进行关于LN诊断和(联合)治疗的调查研究。

结果

58名医生完成了调查。总体而言,在青少年增殖性LN的建议诊断和管理方面存在相当大的异质性。通过24小时尿收集或随机尿(蛋白肌酐比值)评估的尿蛋白排泄增加以及估计肾小球滤过率降低被指定为诊断增殖性LN和监测治疗的重要指标。通常建议使用皮质类固醇进行诱导和维持治疗,最常与霉酚酸酯(MMF)或环磷酰胺(CP)联合使用作为类固醇节约型免疫抑制剂。在III级LN的诱导治疗中,MMF明显优于CP,而在IV级LN中,CP和MMF被同等推荐。MMF最常被推荐用于与口服皮质类固醇联合的维持治疗,并在缓解后分别持续至少3年和1年。羟氯喹被广泛接受为一种辅助措施,其次是在动脉高血压和/或蛋白尿病例中使用肾素 - 血管紧张素系统抑制剂。

结论

德国和奥地利的大多数儿科风湿病学家和肾病学家建议使用皮质类固醇,最常与MMF或CP联合使用,用于治疗儿童增殖性LN。相当大的反应异质性支持在儿科风湿病学家和肾病学家之间制定青少年增殖性LN的靶向治疗方案的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3843/9072733/cc64ccfcc85a/fped-10-851998-g001.jpg

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