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Kidney Week

Abstract: FR-PO196

Scleroderma Renal Crisis: A Nephrologist's Enigmatic Therapeutic Dilemma

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Onuigbo, Macaulay A., University of Vermont Larner College of Medicine, Burlington, Vermont, United States
  • Krant, Nicholas, University of Vermont Larner College of Medicine, Burlington, Vermont, United States
  • Bair, Steven, University of Vermont Medical Center, Burlington, Vermont, United States
Introduction

Scleroderma Renal Crisis (SRC), occuring in 5-20% of patients with systemic scleroderma, typically presents with hypertensive urgency, acute kidney injury (AKI), microangiopathic hemolytic anemia and renal thrombotic microangiopathy. SRC is one of the very few times when AKI treatment calls for aggressive ACE inhibition, especially with Captopril. We describe fatal SRC, more than 20 years after initial diagnosis, AKI requiring hemodialysis despite escalated oral Captopril therapy for hypertension. She died 10 days after starting hemodialysis.

Case Description

A 71-year-old female with scleroderma since 2000 and treated intracranial oligodendroglioma since 2010 was admitted in late December 2022, with abdominal pain and decreased appetite. Blood pressure was 186/109 mm Hg, pulse 108/min and pulse ox 94% on room air. She was diagnosed with SRC – hypertension, microangiopathic hemolytic anemia, elevated LDH, +1 schistocytes, AKI with more than doubled creatinine to 2.21 mg/dL, UACR 935 ug/mg, >50 RBC/HPF and >50 WBC/HPF. Escalated oral Captopril was initiated. PLASMIC score was 4 points, low probability for TTP. ADAMTS13 was 53%, nondiagnostic. Hypertension was controlled on 137.5 mg Captopril TID + Nifedipine 60 mg BID. She was discharged, after 3 weeks, with stabilized blood pressure, stable creatinine at 4 mg/dL, and urine output of 1 L/day.

Six days later, she was readmitted with worsening hypoxia, hyperkalemia 5.9 mmol/L, and creatinine of 5.12 mg/dL, which improved with fluid resuscitation. Potassium improved with oral sodium zirconium cyclosilicate. Kidney biopsy demonstrated thrombotic microangiopathy. She was treated for hypotension.Intermittent hemodialysis was started for worsening AKI and uremic encephalopathy. The patient passed away nine days after starting hemodialysis.

Discussion

SRC is an early complication of systemic sclerosis (SS), usually within one year of diagnosis. This case corroborates the difficulty in treating SRC-induced hypertension and resultant kidney damage. Our patient was atypical, with SRC occurring more than 20 years after diagnosis, hence the need for heightened vigilance. SRC was previously the leading cause of death in SS; the prognosis improved significantly with the introduction of ACE inhibitors. Despite adequate management of hypertension, our patient ultimately succumbed to her disease.