Clinical features and long-term outcomes of Chinese patients with scleroderma renal crisis

To investigate the clinical features, treatments, and long-term outcomes of Chinese patients with scleroderma renal crisis (SRC).

We retrospectively reviewed the clinical and laboratory data of 538 patients with systemic sclerosis (SSc) at our center from January 2009 to December 2016, including 29 SRC and 509 SSc without SRC patients. The treatments and long-term outcomes of patients with SRC were also retrospectively analyzed.

The prevalence of SRC was 5.4% in our cohort. Male gender (odds ratio [OR] =4.194 [95% CI 1.494-11.773]), glucocorticoid exposure (OR = 3.666 [1.484-9.056]), pericardial effusion (OR = 11.180 [4.515-27.681]), and myocardial involvement (OR = 7.958 [1.664-38.064]) were associated with an increased risk of development of SRC. Despite the wide use of angiotensin-converting enzyme inhibitors, the permanent dialysis rate of patients with SRC was 48.3%. Sixteen patients died during follow-up, and the estimated 1- and 5-year survival rates of patients with SRC were 62.1% and 47.3%, respectively. Withdrawal of dialysis (5 patients) and myocardial complications (3 patients) were the main causes of death in patients with SRC. Patients with serum creatinine level >500 µmol/L before treatment (log rank test 5.051, P = 0.025) and/or those who needed dialysis at the onset of SRC (log rank test 12.870, P < 0.001) showed poorer prognosis.

SRC is a rare but severe complication in patients with SSc. Male gender, glucocorticoid exposure, pericardial effusion, and myocardial involvement were risk factors in the development of SRC. Withdrawal of dialysis and myocardial complications were the main causes of death in Chinese patients with SRC.