Clinical characteristics and outcomes in good syndrome patients with infectious complications: a retrospective analysis

Good Syndrome (GS) is a rare combined immunodeficiency syndrome characterized by the presence of thymoma, hypogammaglobulinemia, and opportunistic infections. To date, there are limited reports of GS patients concurrent with various infectious diseases. We conducted a retrospective analysis of clinical records from patients with GS at Peking Union Medical College Hospital between January 2015 and December 2024. Data on clinical characters, management and prognosis were systematically collected and analysed. The age of GS patients at diagnosis was 57.8±9.3 years, with 37% being male. AB-type thymoma was the most prevalent subtype (47.6%). During the follow-up period, all patients exhibited various infectious diseases. Respiratory infections were the most common (88.9%) reported complications. The most common bacteria, fungi, and viruses were Pseudomonas aeruginosa (22.2%), Candida species (44.4%), and cytomegalovirus (CMV) (55.6%), respectively. About 44.4% of GS patients were complicated with autoimmune diseases and 7.4% had concurrent other malignancies. Radiotherapy or chemotherapy for thymoma was performed to 25.9% of patients. COX regression analysis model identified two potential risk factors: age at onset (years, P = 0.018, HR = 1.270, 95% confidence interval [1.042- 1.548]), the duration of corticosteroids (GCs) use before infection (days, P = 0.033, HR = 1.049, 95% CI [1.004 - 1.095]). Cluster analysis stratified patients into five subgroups based on immunodeficiency profiles and correlation analysis showed that CMV infection was associated with cellular immune deficiency (P = 0.001), elevated C-reactive protein levels correlated with phagocytic immune deficiency (P = 0.03), and intensive care unit admission was linked to GCs use before infection as well as reduced lymphocyte counts (P = 0.02). GS patients typically suffered from various infectious disease, with respiratory infections being the most prevalent. The most commonly associated bacterial, fungal, and viral pathogens were Pseudomonas aeruginosa , Candida , and CMV respectively. Corticosteroids administration prior to infection onset may correlate with a poor outcome in GS patients complicated by infectious diseases. Not applicable.