IgA-dominant glomerulonephritis with a membranoproliferative pattern of injury

Hum Pathol. 2018 Nov;81:272-280. doi: 10.1016/j.humpath.2018.06.031. Epub 2018 Jul 3.

Abstract

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.

Keywords: Hepatitis; IgA; Infection; Kidney biopsy; Liver failure; Membranoproliferative glomerulonephritis.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Biopsy
  • British Columbia
  • Child
  • Chronic Disease
  • Databases, Factual
  • Diagnosis, Differential
  • Female
  • Glomerulonephritis, IGA / immunology
  • Glomerulonephritis, IGA / mortality
  • Glomerulonephritis, IGA / pathology*
  • Glomerulonephritis, IGA / therapy
  • Glomerulonephritis, Membranoproliferative / immunology
  • Glomerulonephritis, Membranoproliferative / mortality
  • Glomerulonephritis, Membranoproliferative / pathology*
  • Glomerulonephritis, Membranoproliferative / therapy
  • Humans
  • Immunoglobulin A / immunology*
  • Kidney Glomerulus / immunology
  • Kidney Glomerulus / pathology*
  • Liver Diseases / immunology
  • Liver Diseases / mortality
  • Liver Diseases / pathology
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • United States
  • Young Adult

Substances

  • Immunoglobulin A