Abstract
Patients with anti-IFN-γ autoantibodies have impaired IFN-7 signaling, leading to severe disseminated infections with intracellular pathogens, especially nontuberculous mycobacteria. Disease may be severe and progressive, despite aggressive treatment. To address the underlying pathogenic IFN-γ autoantibodies we used the therapeutic monoclonal rituximab (anti-CD20) to target patient B cells. All subjects received between 8 and 12 doses of rituximab within the first year to maintain disease remission. Subsequent doses were given for relapsed infection. We report 4 patients with refractory disease treated with rituximab who had clinical and laboratory evidence of therapeutic response as determined by clearance of infection, resolution of inflammation, reduction of anti-IFN-γ autoantibody levels, and improved IFN-7 signaling.
Original language | English (US) |
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Pages (from-to) | 3933-3939 |
Number of pages | 7 |
Journal | Blood |
Volume | 119 |
Issue number | 17 |
DOIs | |
State | Published - Apr 19 2012 |
ASJC Scopus subject areas
- Hematology
- Biochemistry
- Cell Biology
- Immunology