Anti-interferon-? autoantibody syndrome
Conditions
Interventions
Conventional treatment group:Conventional anti-infective treatment
Rituximab treatment group:Rituximab 375mg/m^2 intravenously 1 time on the basis of conventional treatment
Sponsors
The First Affiliated Hospital of Guangzhou Medical University
Eligibility
Sex/Gender
All
Age
18 Years to 100 Years
Inclusion criteria
Inclusion criteria: 1. Aged 18 years or older; 2. There were common pathogens of AIGAs such as Talaromyces marneffei or non-tuberculous mycobacteria infection, with prolonged course of disease, spread of infection, increased AIGA titer and positive neutralization test (BLI, flow cytometry, Elisa); 3. At the time of enrollment, the participants were in the stable phase of infection, and the neutralization activity of AIGA test was greater than 1:500 or the titer was greater than 2000; 4. Signed the informed consent before the trial, and fully understood the trial content, process and possible adverse reactions.
Exclusion criteria
Exclusion criteria: 1. Participants with other congenital or secondary immunodeficiency; 2. In the acute phase of infection; 3. Participants who have received immunosuppressive therapy such as cyclophosphamide or glucocorticoids within the previous 90 days; 4. Severe participants with secondary infection requiring ICU admission; 5. Blood routine examination at screening: white blood cell count > 10.0×10^9/L; 6. Participants who participated in clinical trials of other drugs within 90 days before medication; 7. According to the investigator's judgment, they were not suitable to participate in the study; 8. Suspected or indeed having a history of alcohol or drug abuse; 9. Those who did not sign informed consent; 10. Participants deemed unsuitable by other investigators.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The incidence of recurrence of infection in participants during the observation period after treatment (1 year); | — |
Secondary
| Measure | Time frame |
|---|---|
| Titer and Duration of Anti-Interferon-Gamma Autoantibodies;Changes in TBNK Cell Counts and Proportions;Changes in myeloid cell Counts and Proportions;Changes in Levels of Cytokines (IL-4, IL-6, IL-10, TNF-a, IFN-?) and Related Indicators;Serum antibody IgG, IgE, IgA, IgM and other indicators;Quality of life (Quality of life and nutritional Status questionnaire) score;Proportion of subjects with SGRQ score improvement >=4 points;Imaging changes of lesion size and number were observed; | — |
Countries
China
Contacts
Public ContactFeng Ye
The First Affiliated Hospital of Guangzhou Medical University
Outcome results
None listed