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2016 ; 95
(22
): e3748
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Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated
Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis
#MMPMID27258503
Jarrot PA
; Chiche L
; Hervier B
; Daniel L
; Vuiblet V
; Bardin N
; Bertin D
; Terrier B
; Amoura Z
; Andrés E
; Rondeau E
; Hamidou M
; Pennaforte JL
; Halfon P
; Daugas E
; Dussol B
; Puéchal X
; Kaplanski G
; Jourde-Chiche N
Medicine (Baltimore)
2016[May]; 95
(22
): e3748
PMID27258503
show ga
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.