IgA-dominant Glomerulonephritis with a Membranoproliferative Pattern of Injury

Immunoglobulin A (IgA)-dominant membranoproliferative glomerulonephritis (MPGN) is a descriptive term for renal biopsies in which differential diagnoses of unusual IgA nephropathy (IgAN), infection-related GN, or other etiologies are considered. We sought to understand clinical and pathologic features of this finding. Native kidney biopsies with IgA-dominant immune deposits and diffuse MPGN features without significant exudative features or subepithelial deposits were retrospectively reviewed. Two groups (n = 27, 33 biopsies) were identified: patients with chronic liver disease and those without. Patients without chronic liver disease (n = 15) were men (73%, age 40) who presented with nephrotic-range proteinuria, hematuria, renal insufficiency, negative serologic studies, and no history of infection. At a median interval of 3 years, 11 had available follow-up information. Three (27%) progressed to end-stage renal disease. One had recurrent IgA-dominant GN in the renal allograft less than 1 year posttransplant. Four of 5 patients with repeat biopsies had persistent IgA-dominant MPGN. Patients with chronic liver disease (n = 12) had similar biopsy findings, but 42% had concurrent infections, some occult. At a median interval of 7 weeks, 8 patients (80% of those with follow-up) had died and 2 were dialysis dependent. In conclusion, IgA-dominant MPGN was seen in 2 clinical cohorts in this study. In patients without chronic liver disease, this appears to represent either a unique clinicopathologic entity with a poorer prognosis than IgAN or an aggressive variant of IgAN. Patients with chronic liver disease often have underlying infection, and regardless of treatment, die within 1 year because of complex medical conditions.