![]() Treatment for AOID and IgG4-RD includes immunosuppression with steroids and steroid sparing biologic agents. Thus, management continues to be a challenge.įurthermore, a concomitant diagnosis of AOID and IgG4-RD has only been described twice in the literature. ĭue to the novelty of both IgG4-RD and AOID, the natural history of these diseases is often described through case report and remain poorly understood. However, since the establishment of unified diagnostic criteria in 2011, we have seen a growing number of IgG4-RD cases reported internationally within the literature. The reported incidence rate of IgG4-RD was estimated to be around 1 case for every 10,000 residents in Japan. As a result, the true prevalence of IgG4-RD is unknown. Immunoglobulin G4-related disease (IgG4-RD) can be misdiagnosed given its clinical presentation can mimic other disease processes including infections, cancer, or other immune mediated conditions. ![]() First described in 2004, this disease predominantly affects patients of Southeast Asian descent and is characterized by reactive dermatoses and persistent, disseminated infections. Acquired, neutralizing autoantibodies inhibit the downstream signaling of interferon (IFN)-gamma causing an increased susceptibility to intracellular pathogens leading to severe disseminated infections, especially nontuberculous mycobacterial (NTM) species. 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.Īnti-interferon-gamma autoantibody-associated immunodeficiency syndrome is an emerging adult onset immunodeficiency (AOID) disease. However, anti-interferon-gamma autoantibody titers were re-sent after immunosuppression was held and returned strongly positive. Original titers of anti-interferon-gamma autoantibodies were falsely negative due to being on immunosuppressive therapy for his IgG4-related disease. He later re-presented with disseminated mycobacterium avium complex infiltration of his joints, bones and prostate. He was managed with immunosuppressive treatment with prednisone, rituximab and cyclophosphamide. ![]() He was eventually diagnosed with IgG4-related disease through excisional nodal biopsy. Case presentationĪ 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. Concomitant diagnoses of these diseases present a diagnostic and management challenge. Few cases have documented a relationship with IgG4-related disease. 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.
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