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Membranous Nephropathy Associated With Immunological Disorder-Related Liver Disease

Maxime Dauvergne Anissa Moktefi 1 Marion Rabant 2 Cécile Vigneau 3 Tomek Kofman 4 Stéphane Burtey 5 Christophe Corpechot 6 Thomas Stehlé 7 Dominique Desvaux 4 Nathalie Rioux-Leclercq 3 Philippe Rouvier 8 Bertrand Knebelmann 9 Jean-Jacques Boffa 10 Thierry Frouget 3 Eric Daugas 11 Mathieu Jablonski Karine Dahan 12 Isabelle Brochériou 13 Philippe Remy 14 Philippe Grimbert 14 Philippe Lang 4 Oliver Chazouilleres Dil Sahali 15 Vincent Audard 14
1 INSERM U955, équipe 21
Service de néphrologie et transplantation, IMRB - Institut Mondor de Recherche Biomédicale
2 CHU Necker - Enfants Malades [AP-HP]
3 CHU Pontchaillou [Rennes]
4 IMRB - Institut Mondor de Recherche Biomédicale
5 VRCM - Vascular research center of Marseille
6 Service d'hépatologie [Saint-Antoine]
7 UPD5 Médecine - Université Paris Descartes - Faculté de Médecine
8 Service de pathologie [CHU Pitié-Salpêtrière]
9 Service de néphrologie adultes [CHU Necker]
10 Remodelage et Reparation du Tissu Renal
11 UMR 1599
12 Service de Néphrologie et Dialyses [CHU Tenon]
13 Service de Pathologie [CHU Tenon]
14 Service de néphrologie [CHU Henri Mondor]
15 Hôpital Henri Mondor
Abstract : The association between membranous nephropathy (MN) and immunological disorder-related liver disease has not been extensively investigated, and the specific features of this uncommon association, if any, remain to be determined.We retrospectively identified 10 patients with this association. We aimed to describe the clinical, biological, and pathological characteristics of these patients and their therapeutic management. The possible involvement of the phospholipase A2 receptor (PLA2R) in these apparent secondary forms of MN was assessed by immunohistochemistry with renal and liver biopsy specimens.The mean delay between MN and liver disease diagnoses was 3.9 years and the interval between the diagnosis of the glomerular and liver diseases was <1.5 years in 5 patients. MN was associated with a broad spectrum of liver diseases including primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH), and primary sclerosing cholangitis (PSC). AIH whether isolated (n = 3) or associated with PBC (n = 2) or PSC (n = 2) was the most frequent autoimmune liver disease. Circulating PLA2R antibodies were detected in 4 out of 9 patients but the test was performed under specific immunosuppressive treatment in 3 out of 9 patients. Seven of the 9 patients with available renal tissue specimens displayed enhanced expression of PLA2R in glomeruli whereas PLA2R was not expressed in liver parenchyma from these patients or in normal liver tissue. The study of immunoglobulin (Ig) subclasses of deposits in glomeruli revealed that the most frequent pattern was the coexistence of IgG1 and IgG4 immune deposits with IgG4 predominating.Detection of PLA2R antibodies in glomeruli but not in liver parenchyma is a common finding in patients with MN associated with autoimmune liver disease, suggesting that these autoantibodies are not exclusively detected in idiopathic MN.
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https://hal-univ-rennes1.archives-ouvertes.fr/hal-02445576
Contributor : Cécile Vigneau <>
Submitted on : Monday, January 20, 2020 - 11:53:57 AM
Last modification on : Wednesday, September 2, 2020 - 3:22:50 AM

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UNIV-RENNES1 | IMRB | UPEC-UPEM | UPEC | UPMC | UNIV-PARIS5 | UNIV-RENNES | UNIV-AMU | SORBONNE-UNIVERSITE | USPC | CNRS | UNIV-PARIS | SU-MEDECINE

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Maxime Dauvergne, Anissa Moktefi, Marion Rabant, Cécile Vigneau, Tomek Kofman, et al.. Membranous Nephropathy Associated With Immunological Disorder-Related Liver Disease. Medicine, Lippincott, Williams & Wilkins, 2015, 94 (30), pp.e1243. ⟨10.1097/MD.0000000000001243⟩. ⟨hal-02445576⟩

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