Case Report

A Case of Early Onset Normotensive Scleroderma Renal Crisis in a Patient with Diffuse Cutaneous Systemic Sclerosis

Authors: Hany G. Bashandy, MD, Jason S. Javillo, MD, Steven R. Gambert, MD

Abstract

Scleroderma renal crisis (SRC) has classically been defined as a new onset of accelerated arterial hypertension associated with a rapid increase in serum creatinine concentration and/or microangiopathic hemolytic anemia. SRC occurs in approximately 20% of patients with systemic sclerosis who have diffuse cutaneous manifestations. In addition, 10% of reported cases of SRC with diffuse cutaneous involvement have normal blood pressures; in the majority of these cases, SRC occurs after treatment with corticosteroids. We describe a patient who presented with an early onset SRC in the setting of diffuse cutaneous systemic sclerosis in evolution without prior accelerated arterial hypertension or corticosteroid use.


Key Points


* Patients with diffuse cutaneous systemic sclerosis can present with normotensive scleroderma renal crisis, early in the course of the disease, even before full appreciation of the nature of their systemic disease.


* The key to management of scleroderma renal crisis is early detection and treatment with angiotensin-converting enzyme inhibitor.


* Delay in the diagnosis and treatment of scleroderma renal crisis can result in irreversible kidney damage and death.

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