IgA-dominant glomerulonephritis with a membranoproliferative pattern of injury

Nicole K. Andeen, J. Ashley Jefferson, Shreeram Akilesh, Charles E. Alpers, Mei Lin Bissonnette, Laura S. Finn, John Higgins, Donald C. Houghton, Neeraja Kambham, Alex Magil, Behzad Najafian, Roberto F. Nicosia, Megan L. Troxell, Kelly D. Smith

Research output: Contribution to journalArticle

1 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)272-280
Number of pages9
JournalHuman Pathology
Volume81
DOIs
StatePublished - Nov 2018

Keywords

  • Hepatitis
  • IgA
  • Infection
  • Kidney biopsy
  • Liver failure
  • Membranoproliferative glomerulonephritis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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    Andeen, N. K., Jefferson, J. A., Akilesh, S., Alpers, C. E., Bissonnette, M. L., Finn, L. S., Higgins, J., Houghton, D. C., Kambham, N., Magil, A., Najafian, B., Nicosia, R. F., Troxell, M. L., & Smith, K. D. (2018). IgA-dominant glomerulonephritis with a membranoproliferative pattern of injury. Human Pathology, 81, 272-280. https://doi.org/10.1016/j.humpath.2018.06.031