PDF(Pubmed)
Abstract：
Adult-onset immunodeficiency (AOID) syndrome due to the presence of anti-interferon gamma antibody (AIGA) is characterized by multiple opportunistic infections. We report a case of a 65-year old healthy woman who suffered from Legionella pneumophila and Mycobacterium intracellulare co-infection with clinical presentation mimicking metastatic lung cancer. She presented with chronic cough and weight loss. Her positron emission tomography scan showed a right upper lobe mass, mediastinal lymphadenopathy and multiple bone lesions. Acid fast bacilli culture of the lung mass and mediastinal lymph node revealed Mycobacterium intracellulare and she improved with prolonged antibiotic. Relapse of disseminated Mycobacterium intracellulare infection occurred 15 months post-treatment and AIGA was positive with functional neutralizing activity on downstream immune pathway. AOID syndrome secondary to AIGA was diagnosed. This case illustrated the importance of high index of suspicion of AOID syndrome and the difficulty of early diagnosis. Further studies on its predictive factors and AIGA-targeted treatment modalities are urgently needed.
摘要：
由于存在抗干扰素γ抗体（AIGA）而导致的成人发作性免疫缺陷（AOID）综合征的特征是多种机会性感染。我们报告了一例65岁的健康女性，该女性患有嗜肺军团菌和细胞内分枝杆菌共同感染并模仿转移性肺癌的临床表现。她出现慢性咳嗽和体重减轻。她的正电子发射断层扫描显示右上叶肿块，纵隔淋巴结肿大和多发性骨病变。肺肿块和纵隔淋巴结的抗酸杆菌培养显示细胞内分枝杆菌，延长抗生素治疗后，她有所改善。治疗后15个月发生播散性分枝杆菌胞内感染复发，AIGA阳性，对下游免疫途径具有功能性中和活性。诊断为继发于AIGA的AOID综合征。该病例说明了高度怀疑AOID综合征的重要性和早期诊断的困难。迫切需要进一步研究其预测因素和AIGA靶向治疗方式。
