Refractory fever of unknown origin: analysis of clinical characteristics of patients with trisomy 8 syndrome and autoimmune diseases
10.3760/cma.j.cn311365-20240521-00136
- VernacularTitle:疑难发热待查：8号染色体三体综合征合并自身免疫病的临床特点分析
- Author:
Wenxin CHEN
1
;
Zhangyufan HE
1
;
Yiting TANG
1
;
Qianqian LIU
1
;
Xian ZHOU
1
;
Lingyun SHAO
1
;
Wenhong ZHANG
1
;
Yan GAO
1
Author Information
1. 复旦大学附属华山医院感染科 上海市传染病与生物安全应急响应重点实验室 国家传染病医学中心，上海 200040
- Publication Type:Journal Article
- Keywords:
Fever of unknown origin;
Trisomy 8;
Autoimmune diseases;
Infections
- From:
Chinese Journal of Infectious Diseases
2024;42(10):597-601
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To conduct a thorough analysis of the clinical characteristics in patients with trisomy 8 syndrome and autoimmune diseases, and to provide a new perspective on the diagnosis and management of the fever of unknown origin (FUO).Methods:Patients who were admitted to Huashan Hospital, Fudan University between July 1st, 2021 and May 1st, 2024 for FUO and subsequently diagnosed with trisomy 8 syndrome with autoimmune diseases were included. In this retrospective cohort study, patients were divided into infection and non-infection group according to the etiological evidence, and the clinical characteristics and treatments were collected and compared between the two groups. Statistical analysis was performed using the Mann-Whitney U test. Results:Among the nine enrolled patients, one case was associated with Behet syndrome (BD) without myelodysplastic syndrome (MDS) and without co-occurring infection, eight cases were associated with MDS, among which six cases had both BD and MDS, one case had allergic pneumonia, and one case had rheumatoid arthritis. Six MDS cases had infections. The C-reactive protein (CRP) level in the infection group was significantly higher than that in the non-infection group(72.39(14.62, 132.70) mg/L vs 3.68(2.30, 10.09) mg/L; Z=1.00, P=0.048). There were no statistically significant differences in other inflammatory markers (such as white blood cell count, platelet count, erythrocyte sedimentation rate, ferritin, and neutrophil CD64 index) between the infection and non-infection groups (all P>0.05). In the infection group, one had bacterial infection, five had fungal infections, including two cases of disseminated aspergillosis, one case of mixed bacterial, fungal, and viral infections, one case of mucormycosis combined with Enterococcus faecalis infection, and one case of pulmonary aspergillosis combined with disseminated Mycobacterium abscessus infection. Among the nine patients, eight patients received immunosuppressive treatment centered on the glucocorticoids and (or) thalidomide, and all six infected patients received the above immunosuppressive treatment based on the anti-infection therapy. Eight of the nine cases were stable and followed up regularly, while one case died due to worsening of illness. Conclusions:Autoimmune diseases associated with trisomy 8 syndrome is rare. In addition to anti-infection treatment, glucocorticoids, thalidomide or other immunosuppressive drugs should be administrated to suppress the inflammatory response in patients with co-infection, and the disease could be well controlled.
