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Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis

Pierre-Andre Jarrot 1, 2, 3 Laurent Chiche 4 Baptiste Hervier 5 Vincent Vuiblet 6 Nathalie Bardin 1, 2, 7 Daniel Bertin 8 Benjamin Terrier 9 Zahir Amoura 10 Emmanuel Andrés 11 Eric Rondeau 12 Mohamed Hamidou 13 Laurent Daniel 14 Jean-Loup Pennaforte 15 Philippe Halfon 16 Eric Daugas 17 Bertrand Dussol 18, 2 Xavier Puéchal 19 Gilles Kaplanski 2, 7, 1 Noemie Jourde-Chiche 1, 2, 3
1 Physiopathologie de l'Endothelium
VRCM - Vascular research center of Marseille
2 VRCM - Vascular research center of Marseille
3 LA CONCEPTION - Hôpital de la Conception [CHU - APHM]
4 CIML - Centre d'Immunologie de Marseille - Luminy
5 DMIIC - Service de Département de médecine interne et immunologie clinique [CHU Pitié-Salpêtrière]
6 MEDyC - Matrice extracellulaire et dynamique cellulaire - UMR 7369
7 Laboratoire d'Immunologie [Hôpital de la Conception - APHM]
8 AMU - Aix Marseille Université
9 Service de Médecine Interne
10 Service de médecine interne [CHU Pitié-Salpétrière]
11 Hôpital Civil de Strasbourg
12 Urgences néphrologiques et transplantation rénale [CHU Tenon]
13 CHU Nantes - Centre hospitalier universitaire de Nantes
14 HCTI - Héritages et Constructions dans le Texte et l'Image
15 Service de Médecine Interne
16 Hôpital Européen [Fondation Ambroise Paré - Marseille]
17 Hôpital Bichat - Claude Bernard
18 Chirurgie urologique et transplantation rénale [Hôpital de la Conception - APHM]
19 Hôpital Cochin [AP-HP]
Abstract : The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome.A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome.The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%).In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options.
Keywords : Immune response Antineutrophil cytoplasmic antibody-associated vasculitis Antineutrophil cytoplasmic autoantibodies Neutrophil extracellular traps
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https://hal-amu.archives-ouvertes.fr/hal-01460623
Contributor : Françoise Dignat-George Connect in order to contact the contributor
Submitted on : Tuesday, February 7, 2017 - 5:26:31 PM
Last modification on : Wednesday, January 26, 2022 - 4:45:38 PM

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Pierre-Andre Jarrot, Laurent Chiche, Baptiste Hervier, Vincent Vuiblet, Nathalie Bardin, et al.. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis. Medicine, Lippincott, Williams & Wilkins, 2016, 95, pp.e3748. ⟨10.1097/MD.0000000000003748⟩. ⟨hal-01460623⟩

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