A Rare Case of Scleroderma Renal Crisis

Abstract

Scleroderma renal crisis is a rare, life-threatening complication of systemic sclerosis. It is characterized by pronounced hypertension, acute kidney injury, and thrombotic microangiopathy. Although its prevalence has decreased over the last decade, and death rates have declined since the introduction of treatment with Angiotensin converting enzyme (ACE)-inhibitors, it remains a challenge due to lack of prevention and rapid progression despite intervention in those who develop renal crisis. We present a 46-year-old female with history of rheumatoid arthritis and scleroderma who presented to the Emergency Department (ED) with complaints of a severe headache associated with nausea and vomiting that started earlier in the day. Patient presented with a blood pressure of 180/103 that did not improve with use of anti-hypertensive medications. Labs were remarkable for anemia and decreased renal function. A CT of the abdomen and pelvis without contrast demonstrated distention of distal esophagus with possible underlying esophageal dysmotility due to degree of distention and dependent areas of tractional bronchiectasis in the bilateral lower lobes that can be seen in the setting of early interstitial lung disease. Treatment with an ACE-inhibitor was initiated, with improvement in blood pressure and resolution of headache. This case demonstrates another rare case and illustrates the importance of rapid recognition and treatment.