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Kidney Week

Abstract: SA-PO040

Hiding in Plain Sight: Normotensive Scleroderma Renal Crisis

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical‚ Outcomes‚ and Trials


  • Chacko, Eric J., Yale School of Medicine, New Haven, Connecticut, United States
  • Shirali, Anushree C., Yale School of Medicine, New Haven, Connecticut, United States

Scleroderma renal crisis is a rare but recognized complication of systemic sclerosis most notably characterized by the presence of abrupt hypertension and progressive renal failure. Here we present a rare case of normotensive scleroderma renal crisis.

Case Description

A 51 year old female with history of systemic sclerosis, pulmonary hypertension, systemic lupus erythematosus presented with worsening lower extremity edema and shortness of breath. She was treated initially with diuretics and antibiotics for volume overload and pneumonia. 13 days later, respiratory status declined and patient became anuric with subsequent rapid creatinine rise from baseline of 1.5mg/dL to 4.98 mg/dL. Urinalysis was significant for 3+ protein and 3+ blood with urine protein-creatinine ratio of 3.2. Serologic workup was negative for the following: ANCA, MPO, PR3, dsDNA, SSA, SSB , C3, C4, and Anti GBM. There was noted positivity for Scl-70 antibody and ANA at 1:1280 in a homogenous pattern. Renal sonogram revealed normal kidney size, patent bilateral renal arteries, normal cortical thickness, and no hydronephrosis. Echocardiogram was obtained which revealed new reduction in ejection fraction to 30%. Blood pressure from admission and until this point remained within systolic range of 120-150mmHg. The patient was transferred to ICU and renal biopsy was obtained which revealed diffuse acute tubular injury and arteriolar vessels with onion skinning and severe luminal narrowing. She was initiated on continuous renal replacement therapy and captopril. Her course was complicated by worsening respiratory failure and septic/cardiogenic shock requiring vasopressors and inotropes. She was unable to consistently tolerate captopril dosing due to subsequent hypotension and eventually passed.


This case illustrates the diagnostic dilemma seen in patients with normotensive scleroderma renal crisis. Although only a small minority of patients with scleroderma renal crisis present without associated hypertension, these patients are at greater risk for delayed diagnosis and exposure to therapies that may worsen underlying disease process (i.e. steroids). Clinicians should retain high clinical suspicion for scleroderma renal crises with normotension and be aware of comorbidities that may be masking underlying hypertension. Furthermore, kidney biopsy should be considered in these patients for definitive diagnosis and management.