系统性硬化症-系统性红斑狼疮-厄里特马托斯重叠综合征肾危象罕见病例:病例报告与文献综述

Şerife Şeyda Zengin Acemoğlu, İpek Türk, Gunay Suleymanlı, Didem Arslan, Kıvılcım Eren Erdoğan, Gülfiliz Gönlüşen
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摘要

我们报告了一名系统性硬化症-系统性红斑狼疮(SSc-SLE)重叠综合征患者,该患者最初被诊断为系统性红斑狼疮。患者接受了肾活检,活检结果被诊断为系统性硬化症肾危象。通过这个病例,我们旨在提高人们对局限性硬皮病肾危象的认识。我们报告了一例罕见的肾危象病例,患者是一名49岁的男性,患有局限性硬皮病-系统性红斑狼疮重叠综合征。患者因主诉手指和脚趾瘀伤、体重减轻和腿部肿胀而到风湿病诊所就诊,于2022年4月被诊断为系统性红斑狼疮。当时肾功能检查正常。确诊约两个月后,他因气短和胸痛到我院急诊科就诊。他的血压为 160/100 mmHg,肌酐(cre)值为 3.8 mg/dl,因此被送入我院。我们对患者的肾脏进行了活检,结果发现了 SSc 肾危象,并伴有明显的硬骨畸形。该患者被诊断为罕见的局限性 SSc 和系统性红斑狼疮并存。局限性 SSc 肾危象是一种罕见病。我们的患者目前仍依赖透析。在出现肾功能衰竭的 SSc-SLE 合并症患者中,可能与 SSc 肾危象、狼疮肾炎或肾危象与狼疮肾炎并存有关。SSc-SLE重叠综合征肾脏受累的鉴别诊断是一项极具挑战性的临床治疗。肾危象很少见,尤其是在局限性 SSc 中。但是,在鉴别诊断时,如果患者有抵抗性高血压和 SSc 皮肤症状,即使 SSc 的其他体征不明显,也必须考虑肾危象。
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A Rare Case of Renal Crisis in Systemic Sclerosis–Systemic Lupus Eritematosus Overlap Syndrome: A Case Report and Literature Review

We present a patient with systemic sclerosis–systemic lupus eritematosus (SSc-SLE) overlap syndrome who was initially diagnosed as SLE. The patient underwent kidney biopsy and was diagnosed as SSc renal crisis as a result of the biopsy. With this case, we aimed to raise awareness about renal crisis in limited scleroderma cases. We present a rare case of renal crisis in a 49-year-old male patient with limited SSc-SLE overlap syndrome. The patient, who applied to the rheumatology clinic with complaints of bruising on the fingers and toes, weight loss, and swelling in the legs, was diagnosed with SLE in April 2022. Renal function tests were normal at that time. About 2 months after this diagnosis, he applied to the emergency department of our hospital with complaints of shortness of breath and chest pain. He was admitted to our clinic because his blood pressure was 160/100 mmHg and his creatinine (cre) value was 3.8 mg/dl. We detected SSc renal crisis as a result of kidney biopsy performed in our patient with marked sclerodactyly. The patient was diagnosed with a rare coexistence of limited SSc and SLE. Renal crisis in limited SSc is a rare condition. Our patient is still dialysis dependent. In SSc-SLE overlapping patients presenting with renal failure, the picture may be associated with SSc kidney crisis, lupus nephritis, or a coexistence of kidney crisis and lupus nephritis. The differential diagnosis of renal involvement of SSc-SLE overlap syndrome is a challenging clinical management. Renal crisis is rare, especially in limited SSc. However, renal crisis must be kept in mind in the differential diagnosis who has resistant hypertension and SSc skin findings even though other signs of SSc are subtle.

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