静脉注射免疫球蛋白治疗系统性硬化症的严重胃肠道受累
Intravenous immunogobulin therapy for severe gastrointestinal involvement in systemic sclerosis.
作者信息
Clark Kristina E N, Etomi Oseme, Denton Christopher P, Ong Voon H, Murray Charles D
机构信息
Centre for Rheumatology and Connective Tissue Diseases, Division of Medicine, Royal Free Hospital Campus, University College London, UK.
Centre for Gastroenterology, Royal Free NHS Foundation Trust, London, UK.
出版信息
Clin Exp Rheumatol. 2015 Jul-Aug;33(4 Suppl 91):S168-70. Epub 2015 Aug 27.
OBJECTIVES
Gastrointestinal (GI) disease is one of the major causes of morbidity in patients with systemic sclerosis (SSc). The most common manifestation of GI disease is oesophageal involvement affecting 70-90% of patients. Severe GI disease is uncommon, but results in symptoms such as early satiety, pseudo-obstruction, weight loss and malnutrition. The pathogenesis is relatively poorly understood, and management focuses on symptomatic control rather than immunomodulation.
METHODS
We describe two cases of patients with SSc myositis overlap syndrome with severe GI involvement who demonstrated improvements in swallowing, early satiety and diarrhoea following the administration of intravenous immunoglobulin (IVIg).
RESUTS
Clinical data related to the two cases were collected by review of medical records.
CONCLUSIONS
GI complications range from mild symptoms to debilitating and life threatening. We propose that IVIg may have an immunomodulatory effect in a subset of patients with SSc myositis overlap syndrome.
目的
胃肠道（GI）疾病是系统性硬化症（SSc）患者发病的主要原因之一。GI疾病最常见的表现是食管受累，影响70-90%的患者。严重的GI疾病并不常见，但会导致早饱、假性肠梗阻、体重减轻和营养不良等症状。其发病机制相对了解较少，治疗主要侧重于症状控制而非免疫调节。
方法
我们描述了两例患有SSc肌炎重叠综合征且严重GI受累的患者，在静脉注射免疫球蛋白（IVIg）后吞咽、早饱及腹泻症状得到改善。
结果
通过查阅病历收集了与这两例病例相关的临床数据。
结论
GI并发症从轻微症状到使人虚弱及危及生命不等。我们提出IVIg可能对一部分SSc肌炎重叠综合征患者具有免疫调节作用。
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