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

Pierre-Andre Jarrot; Laurent Chiche; Baptiste Hervier; Vincent Vuiblet; Nathalie Bardin; Daniel Bertin; Benjamin Terrier; Zahir Amoura; Emmanuel Andrés; Eric Rondeau; Mohamed Hamidou; Laurent Daniel; Jean-Loup Pennaforte; Philippe Halfon; Eric Daugas; Bertrand Dussol; Xavier Puéchal; Gilles Kaplanski; Noemie Jourde-Chiche

doi:10.1097/MD.0000000000003748

Journal articles

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 Service de médecine interne et d'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 CHU 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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Journal articles

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Life Sciences [q-bio]

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https://hal-amu.archives-ouvertes.fr/hal-01460623
Contributor : Françoise Dignat-George <>
Submitted on : Tuesday, February 7, 2017 - 5:26:31 PM
Last modification on : Wednesday, August 19, 2020 - 12:08:19 PM

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

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

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