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Abstract: PO1493

Nephrotic Range Proteinuria due to ANCA-Associated Vasculitis in a Diffuse Systemic Sclerosis Patient: A Rare Presentation

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation


  • Sharma, Pranav, Rutgers University New Brunswick, New Brunswick, New Jersey, United States
  • Khalil, Steve I., Rutgers University New Brunswick, New Brunswick, New Jersey, United States
  • Lebowitz, Jonathan, Rutgers University New Brunswick, New Brunswick, New Jersey, United States

Scleroderma renal crisis (SRC) is a severe complication of SSc and typically presents with new-onset hypertension and a reduction in renal functioning. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare co-occurrence with SSc. We present a rare case of diffuse systemic sclerosis who presented with features of SRC and AAV with nephrotic range proteinuria without significant renal failure.

Case Description

The patient is a 32-year old woman with diffuse systemic sclerosis complicated by pulmonary fibrosis who had been treated with Mycophenolate mofetil but she discontinued treatment in order to conceive. She reports having an uncomplicated pregnancy without proteinuria or hypertension, but she delivered 7 weeks early. After delivery, she began experiencing weakness of her lower extremities and given steroids for suspecting demyelinating neuropathy and restarted treatment with Mycophenolate mofetil. She had improved but few weeks later (3 months after delivery), she was readmitted with hypertensive encephalopathy in setting of suspected scleroderma renal crisis. She also developed a rash and swelling in her legs after her pregnancy. Her urinalysis was remarkable for proteinuria and numerous RBCs and quantification of the urine protein revealed nephrotic range proteinuria (4.6 g/24 hours) but her serum creatinine (0.6 mg/dl) remained normal. She was found to be positive for antinuclear antibodies, rheumatoid factor, myeloperoxidase antibodies, MPO-ANCA (> 8.0 IU/mL). Her complement levels were within normal limits. She underwent a renal biopsy, which revealed an acute necrotizing vasculitis consistent with AAV.


Clinicians should remain vigilant for concomitant autoimmune disorders in patients with scleroderma. In the background of systemic sclerosis, our patient developed AAV with neurologic and renal manifestations. Thus, renal injury, proteinuria or rapid onset of hypertension should not be assumed to be due to scleroderma renal crisis, particularly when there is nephrotic range proteinuria or an active urine sediment.