目的:常见的可变免疫缺陷障碍(CVID)患者可能有胃肠道(GI)受累和感染,人们对此知之甚少。本研究旨在评估临床,内窥镜,和具有胃肠道症状的CVID患者的组织病理学特征,并确定其与感染的相关性。
方法:我们对2000年至2020年在北京协和医院接受内镜检查的21例有胃肠道症状的CVID患者进行了回顾性研究。临床,传染性,内窥镜,并对组织病理学特征进行了重新评估。
结果:慢性腹泻是最常见的胃肠道症状,在我们的CVID队列中观察到95.2%。超过85%的患者体重低和吸收不良。在内窥镜检查中发现了90.5%的患者小肠绒毛萎缩,并且大多数经组织病理学证实。在9例(42.9%)患者中发现了胃肠道感染。其中,7例小肠弥漫性、明显结节性淋巴样增生(NLH)患者在内镜下的感染率明显增高(85.7%vs21.4%,p<0.05),主要与贾第虫和细菌。小肠活检显示95%的患者缺乏浆细胞,60%的患者上皮内淋巴细胞(IEL)增加,但胃肠道感染与非感染组之间没有显着差异。大多数患者经静脉免疫球蛋白和抗感染治疗后好转。
结论:CVID可能涉及胃肠道,尤其是小肠。内镜下明显的NLH可能是CVID患者胃肠道感染的提示。全面的内镜和组织病理学评估可能有助于CVID诊断和潜在合并感染的识别。导致适当的治疗。
OBJECTIVE: Common variable immunodeficiency disorder (CVID) patients may have gastrointestinal (GI) involvement and suffer from infections, which are poorly understood. This study aimed to evaluate the clinical, endoscopic, and histopathological features of CVID patients with GI symptoms and determine their correlation with infections.
METHODS: We performed a retrospective study on 21 CVID patients with GI symptoms who underwent endoscopic examination in Peking Union Medical College Hospital from 2000 to 2020. The clinical, infectious, endoscopic, and histopathological features were reassessed.
RESULTS: Chronic diarrhea was the most prevalent GI symptom, observed in 95.2% of our CVID cohort. Over 85% of patients had low body weight and malabsorption. Small bowel villous atrophy was found in 90.5% of patients under endoscopy and mostly confirmed by histopathology. GI infections were identified in 9 (42.9%) patients. Of these, 7 patients with diffuse and obvious nodular lymphoid hyperplasia (NLH) of small bowel under endoscopy had significantly higher infection rate (85.7% vs 21.4%, p < 0.05), predominantly with Giardia and bacteria. Small bowel biopsies showed 95% of patients lacked plasma cells and 60% had increased intraepithelial lymphocytes (IELs), but not significantly different between GI infection and non-infection group. Most patients improved after intravenous immunoglobulin and anti-infection therapy.
CONCLUSIONS: CVID could involve GI tract, particularly small bowel. Obvious NLH under endoscopy could be a hint for GI infection in CVID patients. Comprehensive endoscopic and histopathological evaluation may be helpful in CVID diagnosis and identification of potential co-infection, leading to proper treatment.