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10.1097/MD.0000000000003748

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suck abstract from ncbi


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pmid27258503      Medicine+(Baltimore) 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; Andres E; Rondeau E; Hamidou M; Pennaforte JL; Halfon P; Daugas E; Dussol B; Puechal X; Kaplanski G; Jourde-Chiche N
  • Medicine (Baltimore) 2016[May]; 95 (22): e3748 PMID27258503show 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.
  • |*Population Surveillance[MESH]
  • |Adolescent[MESH]
  • |Adult[MESH]
  • |Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*complications/diagnosis[MESH]
  • |Biopsy[MESH]
  • |Female[MESH]
  • |France/epidemiology[MESH]
  • |Glomerulonephritis/diagnosis/epidemiology/*etiology/immunology[MESH]
  • |Humans[MESH]
  • |Incidence[MESH]
  • |Kidney/*pathology[MESH]
  • |Lupus Erythematosus, Systemic/*complications/diagnosis[MESH]
  • |Male[MESH]
  • |Retrospective Studies[MESH]


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