IgG4-related endobronchial lung disease and HIV: a case report and review of the literature

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disorder which typically presents as multiorgan infiltration by polyclonal IgG4-positive plasma cells and responds well to immunosuppression such as systemic corticosteroids. The co-occurrence of IgG4-RD among patients infected with HIV is extremely uncommon and has only been described in a few case reports. We hereby present a 75-years old male with well controlled HIV, who presented with endobronchial mass ultimately diagnosed as IgG4-RD. He received multimodal treatment, which included both local bronchoscopic interventions (cryo-ablation and stent insertion) as well as systemic steroids, with an excellent therapeutic response. Our literature review identified 19 additional case reports of IgG4-RD among individuals with HIV, with significant heterogeneity in clinical presentation, organ involvement patterns, level of HIV control, and serum concentrations of IgG4. Most patients received specific therapy, including steroids, with generally favorable outcomes. This report highlights the possible occurrence of IgG4-RD in HIV patients, despite being immunocompromised, and the possibility of safely treating such cases with immunosuppressive medications.