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Abstract: FR-PO141

Suspected Scleroderma Renal Crisis in Patient with Severe Hypertension and AKI

Session Information

  • AKI: Outcomes, RRT
    November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials


  • Sanchez, Saramarina, HCA Florida Citrus Hospital, Inverness, Florida, United States
  • Saroop, Satesh, HCA Florida Citrus Hospital, Inverness, Florida, United States
  • Padala, Smita, HCA Florida Citrus Hospital, Inverness, Florida, United States

Although rare, scleroderma renal crisis is a potentially fatal complication that affects up to 10% of patients with systemic sclerosis. The etiology of scleroderma renal crisis is poorly understood, however it is thought that vascular endothelial injury, vascular thrombosis and arterial narrowing leads to decreased perfusion of the kidneys. This in turn activates the renin-angiotensin-aldosterone system causing severe hypertension.

Case Description

A 67 year old male with history of scleroderma, pulmonary hypertension, pulmonary fibrosis and hyperlipidemia presented with worsening shortness of breath. Initial systolic blood pressure noted to be in the 180s on occasion reaching 220s with diastolic blood pressures in the 100s. Labs significant for BUN 47, creatinine 1.6 (baseline 0.7), GFR 47 (baseline 100s), CRP 1.17, troponin 0.248, platelets 80, LDH 405, haptoglobin < 20 and urinalysis showing protein > 500 mg. Renal ultrasound showed echogenic kidneys consistent with chronic parenchymal disease. Scleroderma renal crisis was suspected and plan was to initiate captopril 6.25 mg q 4 hrs, however medication was non-formulary. Therefore, Enalapril 2.5 mg IV q 4 hrs was ordered and after 1 dose, systolic blood pressures noted to be in the 130s-150s and diastolic blood pressures in the 70s-90s. He was then started on lisinopril 5 mg po qHS and amlodipine 5 mg po daily to control his blood pressure.


The diagnosis of scleroderma renal crisis must be considered in all patients with systemic sclerosis presenting with AKI and accelerated hypertension. Several risk factors for development of scleroderma renal crisis include renal impairment within first 5 years of disease course, scleroderma skin involvement, glucocorticoid therapy within 6 months and autoantibodies to RNA polymerase III. Scleroderma renal crisis is a diagnosis of exclusion and treatment is more likely to be effective when initiated early, therefore prompt recognition significantly improves outcomes and mortality. While the patient initially presented with typical features of scleroderma renal crisis, his hemodynamic stability and rapid response to ACEi makes it unlikely. Once the patients blood pressure was controlled, renal function significantly improved. The patients symptoms more representative of hypertensive emergency in setting of poorly managed hypertension.