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系统性红斑狼疮伴血栓性微血管病的产后肺肾综合征

Postpartum Pulmonary-Renal Syndrome with Thrombotic Microangiopathy in Systemic Lupus Erythematosus.

作者信息

Jha Vijoy Kumar, Kumar M Harish, Akal Ramanjit Singh, Harikrishnan S, Tirumala Narendra Sreekanth

机构信息

Department of Nephrology, Command Hospital Air Force Bangalore, Bengaluru, Karnataka, India.

Department of Rheumatology, Command Hospital Air Force Bangalore, Bengaluru, Karnataka, India.

出版信息

Indian J Nephrol. 2023 Mar-Apr;33(2):128-131. doi: 10.4103/ijn.ijn_78_22. Epub 2022 Jul 16.

DOI:10.4103/ijn.ijn_78_22
PMID:37234428
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10208545/
Abstract

Postpartum pulmonary syndrome as lupus flares in inactive or mild lupus is uncommon. The diagnosis and management of postpartum lupus flare in second pregnancy presenting with crescentic lupus nephritis (LN), secondary thrombotic microangiopathy (TMA), and severe lupus vasculitis in an undiagnosed systemic lupus erythematosus is extremely challenging. Here, in this case report, we present a young lady who presented with postpartum acute kidney injury (AKI) with systemic complaints about 4 weeks post-term uneventful delivery. Renal biopsy was suggestive of crescentic LN with severe lupus vasculitis. The stormy course was further complicated with diffuse alveolar hemorrhage, portal venous thrombosis, TMA, and anuric AKI requiring renal replacement therapy. She received multiple sessions of plasmapheresis, steroid, intravenous immunoglobulin, inj. cyclophosphamide, and started showing improvement after about 6 weeks of presentation.

摘要

产后肺综合征作为非活动期或轻度狼疮的狼疮发作并不常见。在未诊断的系统性红斑狼疮中,第二次妊娠出现新月形狼疮性肾炎(LN)、继发性血栓性微血管病(TMA)和严重狼疮性血管炎的产后狼疮发作的诊断和管理极具挑战性。在此病例报告中,我们介绍了一位年轻女性,她在足月顺产约4周后出现产后急性肾损伤(AKI)并伴有全身症状。肾活检提示为新月形LN伴严重狼疮性血管炎。病情凶险,进一步并发弥漫性肺泡出血、门静脉血栓形成、TMA和无尿性AKI,需要进行肾脏替代治疗。她接受了多次血浆置换、类固醇、静脉注射免疫球蛋白、注射环磷酰胺治疗,在出现症状约6周后开始好转。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1f9/10208545/ea4a113f558d/IJN-33-128-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1f9/10208545/b980c3d8203a/IJN-33-128-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1f9/10208545/6fb926d8a903/IJN-33-128-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1f9/10208545/ea4a113f558d/IJN-33-128-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1f9/10208545/b980c3d8203a/IJN-33-128-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1f9/10208545/6fb926d8a903/IJN-33-128-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1f9/10208545/ea4a113f558d/IJN-33-128-g003.jpg

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