Scleroderma Renal Crisis as First Manifestation of Systemic Sclerosis

Introduction: Scleroderma renal crisis (SRC) is a rare life-threatening complication of scleroderma typically characterized by abrupt onset of severe hypertension and progressive kidney failure. Most cases occur in patients with diffuse scleroderma compared to individuals with limited cutaneous disease. SRC mostly occurs in the first four years of diagnosis of scleroderma but may rarely, be the herald sign of underlying scleroderma. Here, we describe a patient with no prior diagnosis of scleroderma presenting with SRC. Case Description: 51-year-old female with normal renal function, right ventricular dysfunction, severe pulmonary hypertension on home oxygen, who presented to the emergency room with one-week of shortness of breath and orthopnea. She also had a two-year history of intermittent painful ulcers on fingertips. At home, she was on furosemide 20mg daily and recently prescribed a 14-day course of prednisone 40mg daily. On presentation, blood pressure was 171/119, lung with rales, extremities with sclerodactyly and tender ischemic ulcers on fingertips. Laboratory data with serum creatinine (Scr) of 2.7 mg/dl (from baseline of 0.6 mg/dl). Urinalysis with moderate blood,100 red blood cells, microalbumin-creatinine ratio of 478mcg/mg and total urine protein-creatinine ratio of 1.7g. Given clinical features of sclerodactyly, hypertension, and renal failure, SRC was considered. Oral captopril was started. Serologies with positive Anti-Nuclear-Antibody titer >1:1280 in a speckled pattern, positive ribonucleic (RNA) polymerase III antibody, negative topoisomerase I antibody and 61% ADAMTS13 activity. Scr continued to rise, and a renal biopsy was done. Light microscopy showed subacute thrombotic microangiopathy with predominant involvement of the blood vessels, diffuse ischemic glomerular changes and moderate tubulointerstitial scarring consistent with SRC. A skin punch biopsy showed fibrosing dermopathy consistent with scleroderma. She required hemodialysis due to worsening renal function. The patient’s hospital course was complicated by seizure, superior sagittal sinus thrombosis and then cardiac arrest for which she was pronounced dead. Discussion: SRC has a high mortality rate and prompt aggressive care is warranted. Although being a rare presentation, SRC should be considered in clinical situations where patients present with suspected skin features despite no established diagnosis of scleroderma.