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Kidney Week

Abstract: SA-PO798

Primary Atypical Hemolytic Uremic Syndrome Presenting with Raynaud Phenomenon Triggered by COVID-19

Session Information

Category: Genetic Diseases of the Kidneys

  • 1202 Genetic Diseases of the Kidneys: Non-Cystic

Authors

  • El Sharu, Husam, East Carolina University Brody School of Medicine, Greenville, North Carolina, United States
  • Kavcar, Akil Serdar, East Carolina University Brody School of Medicine, Greenville, North Carolina, United States
  • Lee, Tiffany, Eastern Nephrology Associates PLLC, Greenville, North Carolina, United States
  • Grey, Richard Kyle, Eastern Nephrology Associates PLLC, Greenville, North Carolina, United States
  • Provenzano, Anthony, Eastern Nephrology Associates PLLC, Greenville, North Carolina, United States
Introduction

COVID-19 has been linked to atypical hemolytic uremic syndrome (aHUS), although skin manifestations are rarely reported.

Case Description

A 48-year-old female with Mixed connective tissue disease (MCTD) presented after COVID-19 with Raynaud's phenomenon. Blood film showed schistocytes, ADAMTS13 level was 45%, before PLEX therapy. Labs (Coombs, antiphospholipid, CH50, hepatitis, HIV) were unremarkable but she had a positive RNP antibody and ANA with a titer of 1:320. Renal biopsy showed thrombotic microangiopathy and 20% interstitial fibrosis. Genetic testing revealed a heterozygous MCP/CD46 gene mutation. She needed 2 months of hemodialysis, but symptoms, platelets, and creatinine improved with ravulizumab initiation.

Discussion

Our case highlights skin involvement in primary genetic aHUS and the potential association between MCP/CD46 gene mutation and MCTD, which is rarely reported.

Results
Hemoglobin (mg/dL)11
Platelets (mcL)20
Creatinine (mg/dL)8.44
Blood Urea Nitrogen (mg/dL)84
Haptoglobin (mg/dL)22
Lactate dehydrogenase (U/L)5000

Raynaud's Phenomenon

A Global fibrin thrombus
B Acute tubular necrosis
C Segmental sclerosis, adhesions, and GBM corrugation
D Mesangiolysis and increased lamina rara interna.