Scleroderma Renal Crisis Requiring Kidney Replacement Therapy

Introduction: Systemic Sclerosis (SSc) is an autoimmune disease characterized by vascular dysfunction, and fibrosis of the skin and internal organs. Although cutaneous, pulmonary and renal manifestations are most common, every organ system is vulnerable. SSc often presents with acute flares that can be life threatening if not promptly recognized and managed. Case Description: A 73-year-old woman with SSc presented to the hospital with exertional dyspnea and decreased oral intake. Given prior intolerance to mycophenolate and inability to obtain insurance authorization for Tocilizumab, she was only taking prednisone 20mg. Upon presentation, vital signs were within normal limits except a blood pressure of 180/83. Pertinent labs are shown in Table 1. Further results showed a white blood cell count of 12.3x10^3/uL, hemoglobin of 7.0g/dL, proBNP of 38,000pg/mL, phosphate of 9.0mg/dL, anion gap of 22mmol/L, and lactate of 0.6mmol/L. Urinalysis showed moderate blood, 11/HPF RBCs, 300mg/dL of protein, and a specific gravity of 1.014 without casts. Anti-topoisomerase I antibody titers were elevated. The next day, she developed hyperkalemia and hypervolemia requiring placement of a dialysis catheter and urgent hemodialysis (HD). She was started on captopril which was titrated to maximum tolerated dose. Discussion: Despite guideline-directed medical therapy, our patient developed a life threatening Scleroderma Renal Crisis (SRC). Ultimately, renal failure necessitated renal replacement therapy and she was discharged with outpatient HD. Three months later, she requires regular HD, and is compliant with ACE inhibitor, prednisone 10mg, and Tocilizumab. SRC affects 2-15% of patients with SSc. It is not managed with high dose steroids, but with ACE inhibitor uptitration. One study showed that after 45.8 months, 54% of patients developed ESRD and 41% died. Another study found that renal recovery could occur after 3 to 18 months of HD and ACE inhibitor use. Although our patient’s medication regimen was suboptimal, we highlight the importance of raising clinical suspicion and prompt identification of SRC in the outpatient setting. - On Arrival Next Day Creatinine (mg/dL) 9.85 10.17 Blood Urea Nitrogen (mg/dL) 169 176 Potassium (mmol/L) 5.1 5.7 pH 7.25 7.24 Bicarbonate (mmol/L) 8 9