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系统性红斑狼疮合并IgA肾病病变的不典型表现:来自尼泊尔东部的一例罕见病例报告

Unusual presentations in systemic lupus erythematosus with concurrent IgA nephropathy lesion: a rare case report from Eastern Nepal.

作者信息

Pokhrel Popular, Neupane Nitesh, Karna Nitesh Kumar, Pokhrel Gopal, Chapagain Niraj, Niraula Abhas, Yadav Ajay Kumar

机构信息

B.P. Koirala Institute of Health Sciences.

Department of General Practice and Emergency Medicine, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.

出版信息

Ann Med Surg (Lond). 2024 Jul 23;86(10):6140-6144. doi: 10.1097/MS9.0000000000002401. eCollection 2024 Oct.

DOI:10.1097/MS9.0000000000002401
PMID:39359849
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11444602/
Abstract

INTRODUCTION

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organs. While lupus nephritis (LN) is seen in SLE, concurrent IgA nephropathy lesion is rare. Uncommon manifestations like cutaneous ulcers and orbital involvement present diagnostic challenges, and this case from Nepal emphasizes careful diagnostic approach in such cases.

CASE PRESENTATION

A 42-year-old female presented with bilateral lower limb swelling, gum bleeding, and epistaxis. Initial evaluation revealed pancytopenia and suspected renal involvement. Renal biopsy showed IgA nephropathy lesions, but clinical and laboratory findings favored lupus nephritis. Treatment with immunosuppressive agents was initiated. Despite therapy, the patient developed cutaneous ulcers and orbital cellulitis. Decreasing anti-ds DNA levels were noted during the course of treatment.

DISCUSSION

The diagnosis of lupus nephritis in the presence of IgA nephropathy lesions emphasizes the complexity of SLE diagnosis. Treatment with immunosuppressive agents targeting the underlying autoimmune process, yet the development of cutaneous ulcers and orbital cellulitis highlights the importance of timely intervention in managing SLE complications. In resource-limited settings, clinicians should initiate interventions based on clinical and lab findings while awaiting detailed biopsy results.

CONCLUSION

This case highlights diagnostic challenges in SLE and emphasizes the necessity for careful monitoring and timely intervention in managing complications. The interplay between SLE and IgA Nephropathy (IgAN) suggests that SLE may trigger or exacerbate it, complicating disease management. Further exploration is needed to enhance the understanding and management of complex autoimmune disorders like SLE.

摘要

引言

系统性红斑狼疮(SLE)是一种累及多个器官的自身免疫性疾病。虽然狼疮性肾炎(LN)可见于SLE,但同时合并IgA肾病病变的情况较为罕见。皮肤溃疡和眼眶受累等不常见表现带来了诊断挑战,来自尼泊尔的这个病例强调了在此类病例中采取谨慎诊断方法的重要性。

病例介绍

一名42岁女性出现双侧下肢肿胀、牙龈出血和鼻出血。初步评估发现全血细胞减少并怀疑有肾脏受累。肾活检显示IgA肾病病变,但临床和实验室检查结果更倾向于狼疮性肾炎。于是开始使用免疫抑制剂进行治疗。尽管进行了治疗,患者仍出现了皮肤溃疡和眼眶蜂窝织炎。在治疗过程中抗双链DNA水平有所下降。

讨论

在存在IgA肾病病变的情况下诊断狼疮性肾炎凸显了SLE诊断的复杂性。针对潜在自身免疫过程使用免疫抑制剂进行治疗,但皮肤溃疡和眼眶蜂窝织炎的出现凸显了及时干预SLE并发症的重要性。在资源有限的环境中,临床医生应在等待详细活检结果的同时根据临床和实验室检查结果启动干预措施。

结论

本病例凸显了SLE诊断中的挑战,并强调了在管理并发症时进行仔细监测和及时干预的必要性。SLE与IgA肾病(IgAN)之间的相互作用表明SLE可能触发或加重IgAN,使疾病管理复杂化。需要进一步探索以加深对SLE等复杂自身免疫性疾病的理解和管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb16/11444602/f0ce0e149505/ms9-86-6140-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb16/11444602/f0ce0e149505/ms9-86-6140-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb16/11444602/f0ce0e149505/ms9-86-6140-g001.jpg

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Unveiling Ocular Manifestations in Systemic Lupus Erythematosus.系统性红斑狼疮的眼部表现揭秘
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Current treatment of systemic lupus erythematosus: a clinician's perspective.目前系统性红斑狼疮的治疗:临床医生的视角。
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IgA nephropathy in systemic lupus erythematosus patients: case report and literature review.系统性红斑狼疮患者的IgA肾病:病例报告及文献综述
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