期刊
CLINICAL IMMUNOLOGY
卷 132, 期 1, 页码 124-131出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.clim.2009.03.514
关键词
Autoimmunity; Immunodeficiency; NEMO; Enteritis; Colitis; Inflammatory bowel disease; NF-kappa B; IKK gamma
类别
资金
- NIH/NCRR UCSF-CTSI [UL1 RR024131, T32 HD044331, T32 DK007762, DPI OD00329, K24 DK060617]
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.
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