Abstract：
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.
METHODS: 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.
METHODS: 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.
摘要：
背景：抗-干扰素-γ自身抗体相关的免疫缺陷综合征是一种罕见且未得到充分认可的成人发作性免疫缺陷综合征，与严重的机会性感染如播散性非结核分枝杆菌相关。很少有病例记录与IgG4相关疾病的关系。这些疾病的伴随诊断提出了诊断和管理挑战。
方法：一名61岁的东南亚裔人患有肺分枝杆菌复合感染，出现新的皮疹和恶化的淋巴结病。他最终通过切除淋巴结活检诊断为IgG4相关疾病。他接受了强的松免疫抑制治疗，利妥昔单抗和环磷酰胺。后来他重新出现了播散的鸟分枝杆菌复合物渗入他的关节，骨头和前列腺.由于他的IgG4相关疾病正在进行免疫抑制治疗，因此抗干扰素-γ自身抗体的原始滴度为假阴性。然而,在保持免疫抑制并恢复强阳性后,我们再次发送抗-干扰素-γ自身抗体滴度.
结论：本病例回顾了诊断标准，并讨论了治疗成人合并免疫缺陷综合征患者的现有挑战的管理策略。IgG4相关疾病和播散的分枝杆菌鸟复合体感染。
方法：一名61岁的东南亚裔人患有肺分枝杆菌复合感染，出现新的皮疹和恶化的淋巴结病。他最终通过切除淋巴结活检诊断为IgG4相关疾病。他接受了强的松免疫抑制治疗，利妥昔单抗和环磷酰胺。后来他重新出现了播散的鸟分枝杆菌复合物渗入他的关节，骨头和前列腺.由于他的IgG4相关疾病正在进行免疫抑制治疗，因此抗干扰素-γ自身抗体的原始滴度为假阴性。然而,在保持免疫抑制并恢复强阳性后,我们再次发送抗-干扰素-γ自身抗体滴度.
结论：本病例回顾了诊断标准，并讨论了治疗成人合并免疫缺陷综合征患者的现有挑战的管理策略。IgG4相关疾病和播散的分枝杆菌鸟复合体感染。
