Laboratory of Clinical Immunology and Microbiology, Bethesda, Maryland, USA.
Biostatistics Research Branch, Division of Clinical Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, USA.
Clin Infect Dis. 2020 Jun 24;71(1):53-62. doi: 10.1093/cid/ciz786.
The natural history of anti-interferon-γ (IFN-γ) autoantibody-associated immunodeficiency syndrome is not well understood.
Data of 74 patients with anti-IFN-γ autoantibodies at Srinagarind Hospital, Thailand, were collected annually (median follow-up duration, 7.5 years). Annual data for 19 patients and initial data for 4 patients with anti-IFN-γ autoantibodies at the US National Institutes of Health were collected (median follow-up duration, 4.5 years). Anti-IFN-γ autoantibody levels were measured in plasma samples.
Ninety-one percent of US patients were of Southeast Asian descent; there was a stronger female predominance (91%) in US than Thai (64%) patients. Mycobacterium abscessus (34%) and Mycobacterium avium complex (83%) were the most common nontuberculous mycobacteria in Thailand and the United States, respectively. Skin infections were more common in Thailand (P = .001), whereas bone (P < .0001), lung (P = .002), and central nervous system (P = .03) infections were more common in the United States. Twenty-four percent of Thai patients died, most from infections. None of the 19 US patients with follow-up data died. Anti-IFN-γ autoantibody levels decreased over time in Thailand (P < .001) and the United States (P = .017), with either cyclophosphamide (P = .01) or rituximab therapy (P = .001).
Patients with anti-IFN-γ autoantibodies in Thailand and the United States had distinct demographic and clinical features. While titers generally decreased with time, anti-IFN-γ autoantibody disease had a chronic clinical course with persistent infections and death. Close long-term surveillance for new infections is recommended.
抗干扰素-γ（IFN-γ）自身抗体相关性免疫缺陷综合征的自然病史尚不清楚。
收集了泰国诗里叻医院 74 名抗 IFN-γ 自身抗体患者的数据（中位随访时间为 7.5 年）。还收集了美国国立卫生研究院 19 名患者的年度数据和 4 名患者的初始数据（中位随访时间为 4.5 年）。在血浆样本中测量抗 IFN-γ 自身抗体水平。
91%的美国患者来自东南亚；与泰国（64%）患者相比，美国患者中女性优势更强（91%）。在泰国和美国，脓肿分枝杆菌（34%）和鸟分枝杆菌复合群（83%）分别是最常见的非结核分枝杆菌。皮肤感染在泰国更为常见（P=0.001），而骨骼（P<0.0001）、肺部（P=0.002）和中枢神经系统（P=0.03）感染在美国更为常见。24%的泰国患者死亡，大多数死于感染。在有随访数据的 19 名美国患者中，无人死亡。抗 IFN-γ 自身抗体水平在泰国（P<0.001）和美国（P=0.017）随时间降低，环磷酰胺（P=0.01）或利妥昔单抗治疗（P=0.001）后降低。
泰国和美国的抗 IFN-γ 自身抗体患者具有不同的人口统计学和临床特征。虽然抗体滴度通常随时间降低，但抗 IFN-γ 自身抗体疾病具有慢性临床过程，伴有持续感染和死亡。建议密切长期监测新感染。
