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资源有限中心的孤立性狼疮相关性蛋白丢失性肠病

Isolated Lupus-Associated Protein-Losing Enteropathy in a Resource-Limited Centre.

作者信息

Prabasara Kasun, Sundaresan K T, Rosa Chamith

机构信息

Medicine, Teaching Hospital, Batticaloa, LKA.

Clinical Sciences, Eastern University of Sri Lanka, Batticaloa, LKA.

出版信息

Cureus. 2021 Jun 22;13(6):e15826. doi: 10.7759/cureus.15826. eCollection 2021 Jun.

Abstract

Systemic lupus erythematosus (SLE) is a relatively common autoimmune disease with recently reported cases of lupus-associated protein-losing enteropathy (LUPLE) as an unusual manifestation. It is a well-recognized clinical entity predominantly affecting middle-aged Asian females. LUPLE is diagnosed by exclusion of possible causes for hypoalbuminemia in a patient with positive anti-nuclear antibody (ANA). LUPLE as the first manifestation of SLE is rare but it is a well-recognized complication secondary to SLE. We report a case of a 39-year-old Sri Lankan lady who was investigated for generalized body swelling, pleural effusions, ascites and pericardial effusions due to hypoalbuminemia. Her ANA was positive with speckled pattern and intestinal biopsy samples revealed evidences of chronic inflammatory cell infiltrates in laminapropria. Her investigations were not suggestive of liver diseases, albuminuria or malnutrition. We excluded all possible etiologies for protein-losing enteropathy although gold standard tests to confirm it was not available in our center. In conclusion, LUPLE should be considered as an etiology for all the unexplained protein-losing enteropathies. We suggest to treat LUPLE with prednisolone, hydroxychloroquine (HCQ) followed by steroid-sparing agents such as azathioprine. Prognosis was excellent following appropriate treatment.

摘要

系统性红斑狼疮(SLE)是一种相对常见的自身免疫性疾病,近期有报告称狼疮相关蛋白丢失性肠病(LUPLE)为其一种罕见表现。这是一种公认的临床实体,主要影响中年亚洲女性。LUPLE通过排除抗核抗体(ANA)阳性患者低白蛋白血症的可能病因来诊断。LUPLE作为SLE的首发表现较为罕见,但却是SLE继发的一种公认并发症。我们报告一例39岁斯里兰卡女性病例,该患者因低白蛋白血症导致全身肿胀、胸腔积液、腹水和心包积液而接受检查。她的ANA呈斑点型阳性,肠道活检样本显示黏膜固有层有慢性炎性细胞浸润证据。她的检查未提示肝脏疾病、蛋白尿或营养不良。尽管我们中心没有确认该病的金标准检测方法,但我们排除了蛋白丢失性肠病的所有可能病因。总之,LUPLE应被视为所有不明原因蛋白丢失性肠病的病因之一。我们建议用泼尼松龙、羟氯喹(HCQ)治疗LUPLE,随后使用硫唑嘌呤等激素替代药物。经过适当治疗后,预后良好。

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