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镰状细胞病患者的狼疮性肾炎。

Lupus nephritis in a patient with sickle cell disease.

作者信息

Minocha Vinay, Rana Fauzia

机构信息

Department of Internal Medicine, University of Florida College of Medicine, Jacksonville, USA.

出版信息

Case Rep Hematol. 2013;2013:907950. doi: 10.1155/2013/907950. Epub 2013 Nov 7.

Abstract

Introduction. The diagnosis of systemic lupus erythematosus (SLE) in patients with sickle cell disease (SCD) can be difficult to establish because the musculoskeletal, central nervous system, and renal manifestations are similar in both diseases. In the presented case, we highlight the diagnostic challenge that can evolve in patients with a concurrence of both diseases and we establish the importance of early recognition and treatment of lupus nephritis in patients with SCD. Case Presentation. We present a case of a 31-year-old African American female with sickle-C disease (hemoglobin SC) who was admitted to our hospital with complaints of periumbilical abdominal pain associated with intractable nausea and vomiting, abdominal distension, and worsening lower extremity edema. Urine studies revealed nephrotic range proteinuria and the immunological investigations were consistent with lupus. A renal biopsy revealed focal proliferative lupus nephritis. Conclusion. It is important to consider the presence of a coexisting autoimmune disease in a patient with sickle hemoglobinopathy who displays an atypical and multisystem presentation that is unresponsive to conventional therapies. When a significant kidney disease is present, a renal biopsy is critical in identifying the etiology of a renal abnormality in the setting of coexisting SLE and SCD.

摘要

引言。镰状细胞病(SCD)患者的系统性红斑狼疮(SLE)诊断可能难以确立,因为这两种疾病在肌肉骨骼、中枢神经系统和肾脏表现方面相似。在本病例中,我们强调了这两种疾病并发患者可能出现的诊断挑战,并确立了早期识别和治疗SCD患者狼疮性肾炎的重要性。病例介绍。我们报告一例31岁患有镰状-C病(血红蛋白SC)的非裔美国女性,因脐周腹痛伴顽固性恶心、呕吐、腹胀及下肢水肿加重入院。尿液检查显示肾病范围蛋白尿,免疫学检查结果符合狼疮。肾活检显示局灶增生性狼疮性肾炎。结论。对于患有镰状血红蛋白病且表现出非典型多系统症状且对传统治疗无反应的患者,考虑是否存在并存的自身免疫性疾病很重要。当存在严重肾脏疾病时,肾活检对于在SLE和SCD并存的情况下确定肾脏异常的病因至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/afa0/3838810/e6b9e69443b1/CRIM.HEMATOLOGY2013-907950.001.jpg

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