Scleroderma Renal Crisis is Associated with High Mortality: A Real-World Study from India.

INTRODUCTION

Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis. Since the use of ACE inhibitors in this condition, there has been a significant reduction of mortality rates. However, there is limited data on characteristics and outcomes of SRC from developing countries.

METHOD: This was a single centre, case-control study from India. The records of all patients admitted in the last 5 years were scrutinized, and patients with SRC (as per the updated consensus classification, 2014) were compared with patients of systemic sclerosis who were admitted for other reasons (controls). Disease characteristics, between cases and controls, were compared using chi-squared test, and odds ratios (OR) were calculated. Survival was compared using KaplanMeier statistics.

RESULTS

Ninety-four patients of systemic sclerosis admitted over five-years; among them 15 had SRC. As compared to controls, those with SRC had a significantly higher rates of pericardial effusion (OR 11.7, p=0.02), dilated cardiomyopathy (OR 2.5, p=0.04), myopathy (OR 19.3, p=0.001), taking mediumhigh dose glucocorticoids (OR 7.9, p=0.009) and recent disease onset (OR 39.3, p=0.01). Despite aggressive control of hypertension with ACE inhibitors, 10/12 patients with SRC died. Mean (SD) survival in patients with SRC (11.5, 95% CI 5.7 to 17.6 months) was significantly lower than controls (66.2, 95% CI 58.4 to 73.9 months, p<0.001).

CONCLUSION

In this single-centre study from a developing country, scleroderma renal crisis was associated with a dismal prognosis, despite the use of ACEI. The recent use of medium-high dose glucocorticoids was associated with SRC.