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Abstract: PO1496

Myeloperoxidase-ANCA and Takayasu Arteritis Overlap Syndrome Presenting as Rapidly Progressive Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Gil, Salvador Lopez, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, Mexico City, Mexico
  • Rivas de Noriega, Juan Pablo, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, Mexico City, Mexico
  • Oxlaj, Gibber A., Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, Mexico City, Mexico
  • Toledo, Sebastian Eduardo/e, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, Mexico City, Mexico
  • Garibay Vega, Brian Ricardo, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, Mexico City, Mexico
  • Soto, Virgilia, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, Mexico City, Mexico
Introduction

The spectrum of vasculitides is classified according to the size of the vessels involved and the clinical and histopathological findings. The simultaneous involvement of Takayasu arteritis and myeloperoxidase-ANCA vasculitis is extremely rare

Case Description

A 12-year-old female with no previous medical history. In the last three years the patient developed lower limb claudication and unexplained intermittent fever. In recent weeks she developed edema and oliguria, she was admitted in another hospital where she was started on renal replacement therapy. Large vessel involvement was suspected so a contrast enhanced CT was ordered and was compatible with Takayasu arteritis. The patient was referred to our hospital for evaluation. During nephrology assessment she was found to have acute kidney disease with massive proteinuria (16 g/g), so work up was directed towards rapidly progressive kidney disease. Percutaneous kidney biopsy was performed, and it revealed pauci-immune crescentic glomerulonephritis. ANCA and glomerular basement membrane antibodies were ordered, with a positive MPO-ANCA result. She was then started on IV methylprednisolone pulses and 5 cycles of plasma exchange therapy. After this, rituximab was started on a weekly basis. The patient has not recovered kidney function and is still dependent on hemodialysis therapy, pending to finish the final two rituximab doses.

Discussion

This an extremely rare case which highlights the diagnostic and therapeutic difficulties in patients presenting with overlap clinical and serological features of different forms of systemic vasculitis.

Cellular crescent. Silver methenamine stain