Background: Anti-interferon-gamma autoantibody-associated immunodeficiency syndrome is a rare and underrecognized adult onset immunodeficiency syndrome associated with severe opportunistic infections such as disseminated nontuberculous mycobacterium. Few cases have documented a relationship with IgG4-related disease. Concomitant diagnoses of these diseases present a diagnostic and management challenge. Case presentation: A 61 year old man of Southeast Asian descent with pulmonary mycobacterium avium complex infection presented to our hospital system with a new skin rash and worsening lymphadenopathy. He was eventually diagnosed with IgG4-related disease through excisional nodal biopsy. He was managed with immunosuppressive treatment with prednisone, rituximab and cyclophosphamide. He later re-presented with disseminated mycobacterium avium complex infiltration of his joints, bones and prostate. Original titers of anti-interferon-gamma autoantibodies were falsely negative due to being on immunosuppressive therapy for his IgG4-related disease. However, anti-interferon-gamma autoantibody titers were re-sent after immunosuppression was held and returned strongly positive. Conclusions: This case reviews diagnostic criteria and discusses management strategies with existing challenges in treating a patient with concomitant adult onset immunodeficiency syndrome, IgG4-related disease and a disseminated mycobacterial avium complex infection.
CITATION STYLE
Boyle, S., Hagiya, A., Nguyen, M. V. H., Liebman, H., & Lee, J. S. G. (2022). The unique diagnostic and management challenge of a patient with concomitant anti-interferon-gamma autoantibody associated immunodeficiency syndrome, IgG4-related disease, and treatment refractory, disseminated mycobacterium avium complex infection. Allergy, Asthma and Clinical Immunology, 18(1). https://doi.org/10.1186/s13223-022-00722-x
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