BCG infections occur more frequently in patients with underlying primary immunodeficiency disease (PIDD). In this study, we aimed to evaluate the ratio of PIDD in the patients with BCG infections. Patients with BCG infections were analyzed in a tertiary referral centre in the 2015-2020 period. Forty-seven patients with BCGitis/BCGosis were evaluated; thirty-four (72.3%) had BCGitis, and 13 (27.7%) had BCGosis. Common tissue and organs affected are lymph nodes (57.4%), skin and subcutaneous tissue (48.9%), lungs (23.4%) and liver (17%). PIDD was shown in 26 patients (55.3%), including 92.3% of patients with BCGosis and 41.2% of patients with BCGitis. Ten patients had Mendelian susceptibility to Mycobacterial disease (MSMD) (21.2%), six had predominantly antibody deficiency (PAD) (12.7%), five had severe combined immunodeficiency (SCID) (10.6%), three had CGD (6.3%), and two had CID (4.2%). Mortality was reported in two patients (4.2%) with CID (ZAP70 deficiency (n = 1) and PIK3R1 deficiency (n = 1)). Parental consanguinity (84%), axillary lymphadenopathy (65%), mycobacterial lung disease (42%), hepatomegaly (30%) and growth retardation (19%) were significantly high in patients with PIDD diagnosis. Isolated vaccination site infection was also recorded in patients with PIDD (CID (n = 1), SCID (n = 1), PAD (n = 5)). BCG vaccination should be planned with caution for the cases with suspected PIDD. This study indicates that almost all patients (92.3%) with BCGosis and one in every two patients (41.2%) with BCGitis have an underlying PIDD. Parental consanguinity, axillary lymphadenopathy, mycobacterial lung disease, hepatomegaly and growth retardation (19%) are important clinical features in the differential diagnosis of PIDD.

中文翻译：

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BCG 炎和 BCG 病患者原发性免疫缺陷的鉴别诊断：一项单中心研究

BCG 感染在患有潜在原发性免疫缺陷病 (PIDD) 的患者中更常见。在本研究中，我们旨在评估 BCG 感染患者中 PIDD 的比例。2015-2020 年期间，在三级转诊中心对 BCG 感染患者进行了分析。评估了 47 名 BCG 炎/BCGosis 患者；34 人 (72.3%) 患有 BCG 炎，13 人 (27.7%) 患有 BCG 病。受影响的常见组织和器官是淋巴结 (57.4%)、皮肤和皮下组织 (48.9%)、肺 (23.4%) 和肝脏 (17%)。26 名患者 (55.3%) 出现 PIDD，包括 92.3% 的 BCGosis 患者和 41.2% 的 BCGitis 患者。10 名患者对分枝杆菌病 (MSMD) 具有孟德尔易感性 (21.2%)，6 名患者主要是抗体缺乏 (PAD) (12.7%)，5 名患者患有严重联合免疫缺陷 (SCID) (10.6%)，三个有 CGD (6.3%)，两个有 CID (4.2%)。两名 CID（ZAP70 缺乏（n = 1）和 PIK3R1 缺乏（n = 1））患者 (4.2%) 报告了死亡率。在诊断为 PIDD 的患者中，父母的血缘关系 (84%)、腋窝淋巴结肿大 (65%)、分枝杆菌肺病 (42%)、肝肿大 (30%) 和生长迟缓 (19%) 显着升高。在 PIDD（CID（n = 1）、SCID（n = 1）、PAD（n = 5））患者中也记录了孤立的疫苗接种部位感染。对于疑似 PIDD 的病例，应谨慎计划 BCG 疫苗接种。该研究表明，几乎所有 BCG 病患者 (92.3%) 和每两个 BCG 炎患者中就有一个 (41.2%) 患有潜在的 PIDD。父母血缘关系、腋窝淋巴结肿大、分枝杆菌肺病、