Triple immune impairment in an elderly patient with IgG4-AIP and MGUS complicated by dual severe infections over 3 years: A case report.
Wanman Li, Jiyuan Liang, Yao Xie et al.
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Case in brief
An 82-year-old man with IgG4-related autoimmune pancreatitis and IgGλ monoclonal gammopathy of undetermined significance had markedly elevated serum IgG4 and a monoclonal paraprotein at baseline. Glucocorticoid-based immunosuppression partially improved his pancreatic lesions, but he subsequently developed Staphylococcus hominis bacteremia and active pulmonary tuberculosis. The authors attributed the severe infections to overlapping immune impairment from IgG4-related inflammation, MGUS-associated plasma-cell dysregulation, and treatment-related lymphopenia. Both infections resolved with antibacterial and antituberculosis therapy and adjustment of immunosuppression. During 36 months of follow-up, paraprotein remained stable without progression to multiple myeloma, while IgG4 levels fluctuated independently.
What made this case unusual
The case combined two severe opportunistic infections—S. hominis bacteremia and active pulmonary tuberculosis—in an elderly patient with overlapping IgG4-AIP and IgGλ-MGUS, with serial immune-biomarker monitoring over 36 months.
Diagnostic / clinical pearl
In an older patient with IgG4-AIP and MGUS who develops severe infections during immunosuppression, consider combined disease-related and treatment-related immune impairment, including persistent cellular immunodeficiency.
Why it matters
The authors advise extreme caution with cyclophosphamide in this vulnerable geriatric subgroup.