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儿童狼疮相关蛋白丢失性肠病(LUPLE):一例病例报告及文献综述

Childhood Lupus-Associated Protein-Losing Enteropathy (LUPLE): A Case Report and Review of the Literature.

作者信息

Sumer Cosar Ozlem, Öztürk Hakan, Gezgin Yıldırım Deniz, Küçükali Batuhan, Dalgıç Buket, Eğritaş Gürkan Ödül

机构信息

Faculty of Medicine, Department of Pediatric Gastroenterology and Hepatology, Gazi University, Ankara, Turkey.

Faculty of Medicine, Department of Pediatric Rheumatology, Gazi University, Ankara, Turkey.

出版信息

Pediatr Dev Pathol. 2025 Jun 20:10935266251349494. doi: 10.1177/10935266251349494.

Abstract

Protein-losing enteropathy (PLE) is a rare condition characterized by clinical findings such as edema, ascites, pleural effusion, and diarrhea due to excessive protein loss from the gastrointestinal system. Although systemic lupus erythematosus (SLE) is rare in childhood, PLE can be the first presenting feature; this condition is referred to as lupus-associated protein-losing enteropathy (LUPLE). Protein-losing enteropathy (PLE) is an uncommon condition resulting from excessive protein loss in the gastrointestinal system. Our case shows that PLE can be the initial presentation of SLE, which is a rare manifestation in childhood. PLE, a rare complication of lupus, tends to be more severe in children, and the diagnostic process can be challenging. This case report presents a 7-year-old girl who presented with abdominal distension, generalized edema, chronic diarrhea, and weakness. Despite treatment, the recurrence of symptoms and the addition of new joint findings led to further investigations, which revealed positive anti-dsDNA and low complement levels, resulting in a diagnosis of systemic lupus erythematosus. The patient's clinical condition improved with steroid, azathioprine, and hydroxychloroquine treatments. This case highlights the importance of considering SLE in the differential diagnosis of PLE and underscores the significance of recognizing the rare presentations of childhood lupus.

摘要

蛋白丢失性肠病(PLE)是一种罕见疾病,其特征为因胃肠道系统蛋白质过度丢失而出现水肿、腹水、胸腔积液和腹泻等临床表现。虽然系统性红斑狼疮(SLE)在儿童期罕见,但PLE可能是其首发特征;这种情况被称为狼疮相关性蛋白丢失性肠病(LUPLE)。蛋白丢失性肠病(PLE)是一种因胃肠道系统蛋白质过度丢失导致的罕见病症。我们的病例表明,PLE可能是SLE的初始表现,这在儿童期是一种罕见的表现形式。PLE是狼疮的一种罕见并发症,在儿童中往往更为严重,诊断过程可能具有挑战性。本病例报告介绍了一名7岁女孩,她出现腹胀、全身水肿、慢性腹泻和虚弱症状。尽管进行了治疗,但症状复发以及出现新的关节症状促使进一步检查,结果显示抗双链DNA阳性和补体水平降低,从而诊断为系统性红斑狼疮。患者经类固醇、硫唑嘌呤和羟氯喹治疗后临床状况改善。本病例强调了在PLE鉴别诊断中考虑SLE的重要性,并突出了认识儿童狼疮罕见表现形式的意义。

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