卡介苗(BCG)疫苗接种并发症在先天性免疫错误(IEI)中很常见,这是由于无法清除减毒活的牛分枝杆菌。世界范围内使用各种BCG疫苗株,IEI中俄罗斯BCG毒株疫苗并发症的特征不明确。
在一大群接受俄罗斯BCG毒株疫苗接种的IEI患者中评估BCG感染的风险。
我们评估了778名接受俄罗斯BCG毒株疫苗接种的IEI患者。
共有114人(15%)感染了卡介苗,41(36%)与当地,19(17%)与区域,和54(47%)传播疾病。58%的严重联合免疫缺陷(SCID)患者出现卡介苗感染,82%患有慢性肉芽肿病,50%有先天免疫缺陷,5%合并免疫缺陷,2%与其他IEI。在SCID中,卡介苗感染的中位年龄为4至5个月,慢性肉芽肿病,联合免疫缺陷,与其他IEI组相比,先天免疫缺陷患者的12个月(P<0.005)。我们没有发现特定遗传缺陷的影响,SCID中的CD3+和自然杀伤细胞数,或二氢罗丹明试验刺激指数值在慢性肉芽肿病中对卡介苗感染的风险。即使没有活动性BCG感染,所有SCID患者在SCID诊断时也接受了抗分枝杆菌治疗。相对于局部或区域感染,传播时需要更多的抗分枝杆菌剂(P<0.0001)。114例患者(SCID)中只有1例死于BCG相关并发症(<1%)。
卡介苗感染在接受卡介苗接种的IEI患者中很常见。合理的早期抗分枝杆菌治疗,与抗细胞因子药物联合用于预防移植后炎症综合征,SCID治疗可预防BCG相关死亡。
Bacillus Calmette-Guierin (BCG) vaccination complications are common in inborn errors of immunity (IEI) due to the inability to clear live attenuated Mycobacterium bovis. Various BCG-vaccine strains are used worldwide, and the profile of the Russian BCG strain vaccine complications in IEI is poorly characterized.
To evaluate risks of BCG infection in a large cohort of patients with IEI vaccinated with the Russian BCG strain.
We evaluated 778 patients with IEI vaccinated with the Russian BCG strain.
A total of 114 (15%) developed BCG infection, 41 (36%) with local, 19 (17%) with regional, and 54 with (47%) disseminated disease. BCG infection was seen in 58% of the patients with severe combined immunodeficiency (SCID), 82% with chronic granulomatous disease, 50% with innate immune defects, 5% with combined immunodeficiency, and 2% with other IEI. BCG infection presented at a median age of 4 to 5 months in SCID, chronic granulomatous disease, combined immunodeficiency, and other IEI groups versus 12 months in patients with innate immune defects (P < .005). We found no influence of specific genetic defects, CD3+ and natural killer cell numbers in SCID, or dihydrorhodamine test stimulation index values in chronic granulomatous disease on the BCG-infection risks. All patients with SCID received antimycobacterial therapy at SCID diagnosis even in the absence of active BCG infection. More antimycobacterial agents were required in disseminated relative to local or regional infection (P < .0001). Only 1 of 114 patients (with SCID) died of BCG-related complications (<1%).
BCG infection is common in patients with IEI receiving BCG vaccination. Rational early antimycobacterial therapy, combined with anticytokine agents for posttransplant inflammatory syndrome prevention, and treatment in SCID may prevent BCG-related mortality.