Case Presentation: A 37-year-old female with Systemic Sclerosis (SSc) presented with new-onset elevated blood pressure. She was on medium to high dose Corticosteroids (CS) a month prior to admission. Her significant vitals on admission included a heart rate of 103 and blood pressure of 193/97. Labs showed hemoglobin 9.6, sodium 130, BUN 63, creatinine (Cr) 2.39 with a BUN/Cr ratio of 26. She met the criteria for diagnosis of Scleroderma Renal Crisis (SRC). Conventional treatment was started with captopril 6.25 mg oral three times daily with no response. The patient was transferred to the ICU. There was a decline in her renal function with oliguria and fluid overload. Eventually, she developed progressively worsening thrombocytopenia and anemia, showing smear schistocytes in the blood, elevated Lactate Dehydrogenase, and consumption of haptoglobin likely due to microangiopathic hemolytic anemia related to SRC. Thrombotic Thrombocytopenic Purpura was ruled out, as ADAMTS13 was normal. She showed a dramatic decline in renal and hematologic functions, and renal replacement therapy (RRT) was initiated. However, she decompensated and became hypotensive requiring pressor support, and died of sepsis in a few days.

Discussion: SRC is one of the multi-organ complications of SSc, with a prevalence of 4%–6%; 7%–9% diffuse SSc, and 0.5%–0.6% limited form SSc [1]. SRC is defined as a rapidly progressive renal failure with or without arterial hypertension. Risk factors include the appearance of diffuse skin disease [2], especially with rapid skin progression, the presence of antibody to RNA polymerase III [3], the onset of unexplained anemia, new cardiac disease, and previous use of moderate to high-dose CS [4,5,6].In our patient, given her history of SSc and the presence of accelerated hypertension and worsening Cr, the diagnosis of SRC was made. Our patient also had two known risk factors predisposing her to SRC: early diffuse skin disease and recent use of high dose CS. The presence of these two risk factors increases the risk of SRC double that of patients with SSc without these characteristics [7]. Trang G et al [7] reported that the highest incidence of SRC occurred in those who took medium and high dose CS within the first 4 years of disease and to be 2% among all SSc and 4% among early diffuse disease. In conditions where CS use is unavoidable, it is recommended that the dose of prednisone be limited to < 15 mg/day and to the shortest possible period. It is advised to closely monitor all patients with SSc to initiate timely treatment of SRC. For high-risk patients, daily home blood pressure measurements and quarterly plasma creatinine concentration, and urinary protein-to-creatinine ratio are recommended [8].

Conclusions: The use of CS, one of the mainstays for the treatment of SSc, has been associated with SRC, which is a medical emergency. Hereby, we conclude that there needs to be a criterion similar in urgency to that of SIRS criteria to help clinicians to determine the risk of SRC and direct them to avoid medium to high dose CS. Such a criterion might decrease the incidence of SRC since it is likely that some cases, unfortunately, maybe iatrogenic. It could also potentially guide primary care physicians to refer patients to experts for steroid-sparing medications and simultaneously educate patients regarding avoidance of CS.

IMAGE 1: Diagnostic criteria and Risk factors of Scleroderma Renal Crisis (SRC)

IMAGE 2: Recommended steps prior to prescribing CS in SSc to reduce the risk of SRC