Gros Clothilde, Fogel Olivier, Boudhabhay Idris, Debiais Charlotte, Duong Van Huyen Jean-Paul, Hummel Aurélie, Allanore Yannick, Avouac Jérôme
Service de Rhumatologie, Université de Paris, Hôpital Cochin, Paris, France.
Service de Néphrologie, Université de Paris, Hôpital Necker, Paris, France.
J Scleroderma Relat Disord. 2023 Feb;8(1):NP6-NP10. doi: 10.1177/23971983221099847. Epub 2022 Jun 13.
We report the case of a patient followed for a mixed connective tissue disease with signs of systemic sclerosis and systemic lupus, who presented an acute renal failure with severe neurological symptoms (confusion, obnubilation) and hypertension. The distinction between scleroderma renal crisis and lupus nephritis was challenging and hence, the decision to use or not high dose of corticosteroids. Kidney biopsy was of major importance for the diagnosis and therapeutic strategy. The diagnosis of neurological symptoms was also made difficult given the clinical presentation and the results of imaging. Neurolupus, malignant hypertension, or posterior reversible encephalopathy syndrome were the evoked diagnosis.
我们报告了一例患有混合性结缔组织病且有系统性硬化症和系统性红斑狼疮体征的患者,该患者出现急性肾衰竭,并伴有严重的神经症状(意识模糊、嗜睡)和高血压。硬皮病肾危象与狼疮性肾炎的鉴别具有挑战性,因此,决定是否使用大剂量皮质类固醇。肾活检对诊断和治疗策略至关重要。鉴于临床表现和影像学结果,神经症状的诊断也很困难。诱发的诊断包括神经狼疮、恶性高血压或后部可逆性脑病综合征。