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Membranous Nephropathy Associated With Immunological Disorder-Related Liver Disease: A Retrospective Study of 10

Maxime Dauvergne1, Anissa Moktefi, Marion Rabant

  • 1From the Service de Néphrologie et Transplantation (MD, TK, TS, PR, PG, PL, DS, VA), Groupe Hospitalier Henri-Mondor/Albert-Chenevier, Centre de référence maladie rare Syndrome Néphrotique Idiopathique, Institut Francilien de recherche en Néphrologie et Transplantation (IFRNT), DHU VIC (Virus-Immunité-Cancer), AP-HP (Assistance Publique-Hôpitaux de Paris), Université Paris Est Créteil (UPEC), Créteil, France; Equipe 21 (MD, AM, TK, TS, DD, PG, PL, DS, VA), INSERM Unité 955, Université Paris Est Créteil, Créteil, France; Département de Pathologie (AM, DD), Groupe Hospitalier Henri-Mondor/Albert-Chenevier, AP-HP, UPEC, Créteil, France; Service d'Anatomie pathologique (MR), Hôpital Necker - Enfants Malades, AP-HP, Université Paris Descartes, Paris, France; CHU Pontchaillou (CV, TF), Service de Néphrologie, Université de Rennes, Rennes, France; Service de Néphrologie et de Transplantation Rénale (SB), Hôpital de la Conception, AP-HM, Aix-Marseille Université, Marseille, France; Centre de Référence Maladies Rares des Maladies Inflammatoires des Voies Biliaires & Service d'Hépatologie (CC, OC), Hôpital Saint-Antoine, Centre de Référence Maladies Rares des Maladies Inflammatoires des Voies Biliaires & Service d'Hépatologie; Inserm, UMR-S 938, Centre de Recherche Saint-Antoine; AP-HP, Sorbonne Universités, Université Pierre et Marie Curie (UPMC), Paris, France; CHU Pontchaillou (N R-L), Service d'Anatomie Pathologie, Université de Rennes, Rennes, France; Service d'Anatomie pathologique (PR), Hôpital de la Pitié-Salpétrière, AP-HP, UPMC, Paris, France; Service de Néphrologie et Dialyse (BK), Hôpital Necker-Enfants Malades, AP-HP, Université Paris Descartes, Paris, France; Service de Néphrologie et Dialyses (J-J B, KD), Hôpital Tenon, AP-HP, UMPC, Paris, France; Service de Néphrologie (ED, MJ), Groupe hospitalier Bichat/Claude-Bernard, AP-HP, Université Paris Diderot, Paris, France; Service d'Anatomie pathologique (IB), Hôpital Tenon, AP-HP, UPMC, Paris, France.

Medicine
|July 30, 2015
PubMed

Insights

Membranous nephropathy (MN) can occur with autoimmune liver diseases. Phospholipase A2 receptor (PLA2R) antibodies were found in kidney tissue, not liver, suggesting MN isn't always idiopathic.

Area of Science:

  • Nephrology
  • Hepatology
  • Immunology

Background:

  • The link between membranous nephropathy (MN) and autoimmune liver diseases is poorly understood.
  • Specific clinical, biological, and pathological features of this association require further investigation.

Purpose of the Study:

  • To characterize patients with co-occurring MN and autoimmune liver disease.
  • To investigate the role of phospholipase A2 receptor (PLA2R) in these cases.

Main Methods:

  • Retrospective analysis of 10 patients with MN and autoimmune liver disease.
  • Immunohistochemistry on renal and liver biopsy specimens to detect PLA2R.
  • Analysis of immunoglobulin (Ig) subclass deposits in glomeruli.

Main Results:

  • Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) were associated liver diseases.
  • PLA2R expression was enhanced in glomeruli but absent in liver parenchyma.
  • Coexistence of IgG1 and IgG4 deposits, predominantly IgG4, was common in glomeruli.

Conclusions:

  • PLA2R antibodies in glomeruli, but not liver, are common in MN associated with autoimmune liver disease.
  • This suggests PLA2R-related MN may occur secondary to autoimmune liver conditions, not exclusively idiopathic MN.

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