Efficacy of Bortezomib for Treating Anti-Interferon-Gamma Autoantibody-Associated Adult-Onset Immunodeficiency Syndrome
Issued Date
2024-04-15
Resource Type
ISSN
10584838
eISSN
15376591
Scopus ID
2-s2.0-85190334243
Pubmed ID
37947190
Journal Title
Clinical Infectious Diseases
Volume
78
Issue
4
Start Page
1033
End Page
1042
Rights Holder(s)
SCOPUS
Bibliographic Citation
Clinical Infectious Diseases Vol.78 No.4 (2024) , 1033-1042
Suggested Citation
Angkasekwinai N., Suputtamongkol Y., Tantibhedhyangkul W., Onlamoon N., Phoompoung P., Pithukpakorn M., Karuphong E., Pusuwan P., Angkasekwinai P. Efficacy of Bortezomib for Treating Anti-Interferon-Gamma Autoantibody-Associated Adult-Onset Immunodeficiency Syndrome. Clinical Infectious Diseases Vol.78 No.4 (2024) , 1033-1042. 1042. doi:10.1093/cid/ciad676 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/98082
Title
Efficacy of Bortezomib for Treating Anti-Interferon-Gamma Autoantibody-Associated Adult-Onset Immunodeficiency Syndrome
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Abstract
Background. Currently, there is no effective treatment for adult-onset immunodeficiency (AOID) syndrome with anti-interferon-gamma autoantibodies (anti-IFN-γ-auto-Abs). This study aimed to investigate the effectiveness of bortezomib (BTZ) for decreasing anti-IFN-γ-auto-Abs. Methods. A pre- and post-intervention study was conducted from February 2017 through June 2019 at Siriraj Hospital (Bangkok, Thailand). Five patients were invited to receive once-weekly BTZ (1.3 mg/m2 body surface area) subcutaneously for 8 weeks followed by oral cyclophosphamide (1 mg/kg/d) for 4 months. The primary outcomes were the difference in antibody level at 8 and 48 weeks compared with baseline and the incidence of serious adverse events (AEs). The secondary outcome was the occurrence of opportunistic infections (OIs) during the 72 weeks after starting BTZ. Results. The median patient age was 46 years (range, 34-53). All patients had 3-5 OIs prior to enrollment. All patients were receiving antimycobacterial agents for treatment of nontuberculous mycobacterial infection at enrollment. There was no significant difference in the mean optical density of auto-Abs at 8 weeks (3.73 ± 0.72) or 48 weeks (3.74 ± 0.53) compared with baseline (3.84 ± 0.49; P = .336 and P = .555, respectively). However, after serum dilution, the antibody titer nonsignificantly decreased 8-16 weeks after BTZ initiation (P = .345). Ten OIs were observed 24-72 weeks after BTZ initiation. Conclusions. Treatment with BTZ followed by cyclophosphamide yielded no significant decrease in antibody titer levels, and 10 OIs were observed during 24-72 weeks of BTZ treatment. No serious AEs were observed. Combining rituximab with BTZ is likely necessary to prevent generation of new autoantibody-producing plasma cells.