Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis.
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.
From the Department of Internal Medicine and Clinical Immunology (PAJ, GK), AP-HM Hôpital de La Conception; Inserm UMR-S 1076 Vascular Research Center of Marseille (PAJ, GK, NJ-C), Aix-Marseille Université; Department of Internal Medicine (LC, PH), Hôpital Européen de Marseille, Marseille; Reference Center for Systemic Lupus Erythematosus (BH, ZA), Department of Internal Medicine, AP-HP Hôpital Pitie-Salpêtrière, Paris; Department of Pathology (LD), AP-HM Hôpital de La Timone, Aix-Marseille Université; Department of Pathology (VV), Paul Bouin Laboratory, Reims, France; Laboratory of Immunology (NB, DB), AP-HM Hôpital de la Conception, Aix-Marseille Université, Marseille; French Vasculitis Study Group (BT, XP), Department of Internal Medicine, National Reference Center for Necrotizing Vasculitis, AP-HP Hôpital Cochin, University Paris-Descartes; Department of Internal Medicine (EA), Hôpital Civil, Strasbourg; Department of Nephrology (ER), AP-HP Hôpital Tenon, Paris; Department of Internal Medicine (MH), Hôpital Hôtel-Dieu, Nantes; Department of Internal Medicine (J-LP), Hôpital Robert Debré, Reims Université, Reims; Groupe Coopératif sur le Lupus Rénal (ED), Department of Nephrology, AP-HP Hôpital Bichat, Paris; and Department of Nephrology (BD, NJ-C), AP-HM Hôpital Conception, Aix-Marseille Université, Marseille, France.
Pierre-Andre
PA
Jarrot
Laurent
L
Chiche
Baptiste
B
Hervier
Laurent
L
Daniel
Vincent
V
Vuiblet
Nathalie
N
Bardin
Daniel
D
Bertin
Benjamin
B
Terrier
Zahir
Z
Amoura
Emmanuel
E
Andrés
Eric
E
Rondeau
Mohamed
M
Hamidou
Jean-Loup
JL
Pennaforte
Philippe
P
Halfon
Eric
E
Daugas
Bertrand
B
Dussol
Xavier
X
Puéchal
Gilles
G
Kaplanski
Noemie
N
Jourde-Chiche
D000293:Adolescent; D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy; D005260:Female; D005602:France / Q000453:epidemiology; D005921:Glomerulonephritis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000276:immunology; D006801:Humans; D015994:Incidence; D007668:Kidney / Q000473:pathology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis; D008297:Male; D011159:Population Surveillance; D012189:Retrospective Studies; D055815:Young Adult