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Abstract: TH-PO667

Lupus Nephritis Complicated by Atypical Hemolytic Uremic Syndrome and Heterozygous Deletion in CFHR1-CFHR3 Successfully Treated with Ravulizumab

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Amin, Sahar, The University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, United States
  • Wall, Barry M., The University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, United States
  • Bhatt, Dhirisha, The University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, United States
  • Gyamlani, Geeta G., The University of Tennessee Health Science Center College of Medicine, Memphis, Tennessee, United States
Introduction

Atypical hemolytic uremic syndrome (aHUS) involves unchecked activation of the alternative pathway leading to deleterious action of complement on the endothelium. Failure to control complement activation is often due to inherited or acquired deficiencies of complement regulatory proteins, including Factor I, Factor H, Membrane Cofactor Protein, and CD46.

Case Description

21 year old female with history of SLE presented with acute kidney injury (AKI) with an active urine sediment. Kidney biopsy showed diffuse lupus nephritis class IV and thrombotic microangiopathy (TMA). She was started on mycophenylate (MMF) 1g bid which she received for 2 wk, but was off for 4 wk ,as she was not able to fill her medications, leading to admission for worsening AKI.

Laboratory: hemoglobin 6.1gm/dl, platelet 185,000/ul, serum creatinine 7.25 mg/dl, UPCR 3.5 g/g, low C3 (57mg/dl), LDH 472 IU/l, haptoglobin 36 mg/dl, 2-3 schistocytes noted on blood smear. She was Induced with solumedrol and Myfortic 720 mg BID. Five sessions of plasmapheresis (PLEX) were done due to TMA biopsy findings. She developed microangiopathic hemolytic anemia (MAHA), severe thrombocytopenia, tonic-clonic seizures, and required hemodialysis. ADAMTS 13 level was normal and anti-phospholipid studies were negative. Given the high suspicion of atypical HUS, ravulizumab was added to her regimen. Hemoglobin and platelet count normalized after 2 weeks. She remained dialysis dependent for 6 weeks. Renal function improved and serum creatinine has remained stable at 3.0 mg/dl off dialysis. Genetic testing revealed compound heterozygosity for exon 2-6 deletion in CFHR1 and exon 1-6 deletion on CFHR3.

Discussion

The coexistence of atypical HUS with mutations in complement regulatory proteins and lupus nephritis is rare. Our patient had a successful outcome with addition of ravulizumab. Initial treatment with plasma exchange and conventional therapy with MMF and glucocorticoids did not improve MAHA, thrombocytopenia, or kidney function.