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Abstract: FR-PO677

ANCA-Systemic Lupus Erythematosus Overlap Syndrome: Case Series

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Paliwal, Nitpriya, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Coppock, Gaia M., University of Pennsylvania, Philadelphia, Pennsylvania, United States
Introduction

Systemic lupus erythematosus (SLE) is characterized by development of autoantibodies against nuclear components. ANCA-associated vasculitides (AAV) are characterized by a small-sized vessel vasculitis & ANCA positivity. SLE & AAV are rarely associated as vasculitis may occur during SLE but rarely fits AAV classification criteria. A clinical entity that fulfills criteria for both SLE & AAV was first described in 2008.

Case Description

All 3 patients presented with hematuria, proteinutia & AKI.

Case 1: 71 y/o F. Renal biopsy revealed glomerulonephritis (GN) with dominant IgA deposits and crescents. P-Anca titers were 44. Treated with steroids with improvement & maintained on MMF for 6 months with complete remission. She had a recurrence 6 years after stopping all therapy & repeat renal biopsy revealed crescentic GN with immune complex deposits & near full-house pattern on immunofluorescence (IF). P-ANCA remained positive. She was treated with steroids and started on MMF with her creatinine now stable at 1.

Case 2: 69 y/o F with high ANA titers (1:10240) & + MPO (57). Renal biopsy showed crescentic GN with full house staining on IF & intramembranous & subepithelial immune-type deposits on electron microscopy (EM). Treated with steroids & Cytoxan, followed by maintenance therapy with azathioprine & prednisone. Currently off maintenance therapy for 6 months with stable renal function.

Case 3: 39 y/o F with high ANA titers (1:5120), normal complements ,elevated MPO (66) & anti-ds DNA of 344. Prelim biopsy results revealed marked endocapillary cellularity with few crescents. Treated with solumedrol x3 followed by MMF. Final biopsy report showed full house IF & diffuse crescentic GN, so her treatment was switched to Cytoxan & steroid taper with clinical improvement.

Discussion

We discuss 3 cases of overlap with different presentations & approaches to treatment. Incidence of SLE & ANCA vasculitis overlap syndrome has increased over the last decade. The largest case series to our knowledge was of 8 patients reviewed between 1995-2014. The low number of cases prompts the question of underdiagnosis & significance of ANCA positivity in lupus nephritis. We recommend that ANCA testing be performed in all patients with lupus nephritis who have crescents on biopsy to test for overlap syndrome. Further studies are needed to identify the standard approach to treatment or if treatment should be tailored to individual patient presentation.