%0 Journal Article
%T 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.
%A Boyle S
%A Hagiya A
%A Nguyen MH
%A Liebman H
%A Lee JSG
%A Boyle S
%A Hagiya A
%A Nguyen MH
%A Liebman H
%A Lee JSG
%A Boyle S
%A Hagiya A
%A Nguyen MH
%A Liebman H
%A Lee JSG
%J Allergy Asthma Clin Immunol
%V 18
%N 1
%D Sep 2022 9
%M 36085248
暂无%R 10.1186/s13223-022-00722-x
%X <B>BACKGROUND: </B>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.<BR><B>METHODS: </B>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.<BR><B>CONCLUSIONS: </B>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.