A Critical Care Standpoint in the Diagnosis of Scleroderma Renal Crisis

Typical or atypical presentations of rare diseases may be confounded by co-morbidities in critically-ill patients. It is imperative to diagnose and treat appropriately, despite this difficulty. Scleroderma renal crisis mimics many other conditions, and can be potentially fatal if not caught early enough. Particularly, in critically-ill patients with multiple pathologies, it can be difficult to distinguish scleroderma renal crisis from other diseases, such as thrombotic thrombocytopenic purpura (TTP), hypertensive emergency, posterior reversible encephalopathy syndrome (PRES), or atypical hemolytic uremic syndrome (HUS). Herein, a patient who presented with encephalopathy and seizures was initially treated for thrombotic thrombocytopenic purpura, but was ultimately diagnosed with scleroderma renal crisis. Given her numerous laboratory abnormalities, such as thrombocytopenia, hemolytic anemia, kidney and liver dysfunction, and elevated inflammatory markers, various differentials were considered. During her hospitalization, she suffered a cardiac arrest, seizures, nosocomial infections and worsening kidney disease requiring dialysis, making the final diagnosis of scleroderma renal crisis a diagnosis of exclusion. Subsequently, the management of a patient with multiple co-morbidities and confounding laboratory abnormalities difficult to treat. This article highlights these intricacies and formulates the thought process behind the diagnosis of Scleroderma Renal Crisis.