Pensieri Maria Vittoria, Pulvirenti Federica, Schiepatti Annalisa, Maimaris Stiliano, Lattanzio Salvatore, Quinti Isabella, Klersy Catherine, Corazza Gino Roberto, Biagi Federico
a Coeliac Centre/First Department of Internal Medicine , Fondazione IRCCS Policlinico San Matteo University of Pavia , Pavia , Italy.
b Department of Molecular Medicine , University of Rome Sapienza , Rome , Italy.
Scand J Gastroenterol. 2019 Feb;54(2):164-168. doi: 10.1080/00365521.2019.1568543. Epub 2019 Apr 21.
Common variable immunodeficiency (CVID) is a primary humoral immunodeficiency characterised by reduced serum levels of immunoglobulins, recurrent infections, autoimmune phenomena and lymphoproliferative disorders. Gastrointestinal symptoms are very common in these patients and a coeliac-like villous atrophy was described in some of them. Since mortality in CVID is much higher than in the general population, our aim was to evaluate mortality rates and clinical predictors of survival in patients with both CVID and duodenal villous atrophy.
Sex, date of diagnosis of villous atrophy, HLA genomic typing, date of death/last follow-up, type of complication were retrospectively collected from medical files. Univariate analysis for each predictor was conducted and Kaplan-Meier curves were generated to evaluate survival.
Twenty-three patients were enrolled (9 females, mean age at diagnosis of villous atrophy 38 ± 13 years) and 8 of them died after a median time of 96 months (25th-75th 60-120 months) corresponding to a mortality rate of 3.9 per 100 person-years (95% CI 1.9-7.7). Mortality was higher in men compared to women (60 vs. 11/1000 person-years), although not statistically significant. Causes of death included onco-haematological disorders and infections.
Although based on a small cohort, our results confirm that patients with CVID and villous atrophy are burdened by a very high mortality mainly due to onco-immunological disorders and infections. Strict follow-up is required in these patients.
普通变异型免疫缺陷（CVID）是一种原发性体液免疫缺陷，其特征为血清免疫球蛋白水平降低、反复感染、自身免疫现象和淋巴增殖性疾病。胃肠道症状在这些患者中非常常见，部分患者还出现了类似乳糜泻的绒毛萎缩。由于CVID患者的死亡率远高于普通人群，我们的目的是评估CVID合并十二指肠绒毛萎缩患者的死亡率及生存的临床预测因素。
回顾性收集病历中的性别、绒毛萎缩诊断日期、HLA基因分型、死亡日期/末次随访日期、并发症类型。对每个预测因素进行单因素分析，并绘制Kaplan-Meier曲线以评估生存情况。
共纳入23例患者（9例女性，绒毛萎缩诊断时的平均年龄为38±13岁），其中8例在中位时间96个月（第25-75百分位数为60-120个月）后死亡，死亡率为每100人年3.9例（95%CI 1.9-7.7）。男性的死亡率高于女性（60对11/1000人年），尽管差异无统计学意义。死亡原因包括肿瘤血液系统疾病和感染。
尽管基于一个小队列，但我们的结果证实，CVID合并绒毛萎缩的患者死亡率极高，主要原因是肿瘤免疫性疾病和感染。这些患者需要严格随访。
