Persistent systemic inflammation and atypical enterocolitis in patients with NEMO syndrome
Persistent systemic inflammation and atypical enterocolitis in patients with NEMO syndrome
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DOI：
10.1016/j.clim.2009.03.514
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发表时间：
2009-07-01
影响因子：
8.6
通讯作者：
Wara, Diane W.
中科院分区：
文献类型：
--
作者：
Cheng, Laurence E.;Kanwar, Bittoo;Wara, Diane W.
尼莫综合征是一种具有免疫性和非免疫性表现的原发免疫缺陷。免疫缺陷是异质性的，表现为体液免疫、先天免疫和细胞免疫缺陷。虽然对免疫缺陷的临床方面的了解越来越多，但对NEMO患者的自身免疫表现知之甚少。因此，我们试图检测一类NEMO综合征患者的全身炎症和肠道病理的血清学标志物。我们观察到5名患者的血沉持续升高，其中2名患者有症状，伴有慢性但不典型的小肠结肠炎。虽然这两名患者的病理损害与急性炎症相一致，但只有通过全身和/或局部糖皮质激素治疗，临床才能持续改善。我们的数据表明，一些NEMO综合征患者表现出类似于全身性自身免疫性疾病的炎症标志物的持续升高，并可能随后发展为非典型的小肠结肠炎。(C)2009 Elsevier Inc.保留所有权利。
The NEMO syndrome is a primary immunodeficiency with immune and non-immune manifestations. The immune deficiency is heterogeneous showing defects in humoral, innate, and cell-mediated immunity. While the clinical aspects of the immunodeficiency are increasingly well understood, little is known about autoimmune manifestations in NEMO patients. We therefore sought to examine serologic markers of systemic inflammation and intestinal pathology in a kindred of patients with the NEMO syndrome. We observed persistent elevation of erythrocyte sedimentation rates in five patients, and two were symptomatic, with a chronic but atypical enterocolitis. Though pathologic lesions in these two patients were consistent with acute inflammation, sustained clinical improvement was only achieved with systemic and/or topical glucocorticoid therapy. Our data suggest that some patients with the NEMO syndrome exhibit persistent elevation of inflammatory markers similar to systemic autoimmune diseases and may subsequently develop an atypical enterocolitis. (C) 2009 Elsevier Inc. All rights reserved.