Risk factors and outcome of Thai patients with scleroderma renal crisis: a disease duration-matched case control...

Data regarding the prevalence, risk factors and outcome of scleroderma renal crisis (SRC) in Asian patients with systemic sclerosis (SSc) are limited.

To determine the prevalence, risk factors and outcomes of SRC in Thai SSc patients.

Medical records of all SSc patients seen at the Division of Rheumatology, Chiang Mai University, Thailand, from January 1990 to December 2015 were retrospectively reviewed. For each SRC case, a disease duration (±1 year) matched control (four SSc patient without SRC for each SRC patient) was identified.

Of 608 SSc patients seen during the study period, 19 SRC cases were identified, resulting in an SRC prevalence of 3.13%, with 76 matched controls. Of the 19 cases, mean ± SD age and median (interquartile range 1-3) disease duration was 56.2 ± 13.8 years and 5 (3-22) months, respectively. Seventeen patients (89.5%) had diffuse cutaneous SSc. Twelve patients (63.2%) had hypertensive renal crisis and seven (36.8%) had normotensive renal crisis. Multivariate conditional logistic regression analyses showed that digital gangrene (adjusted odd ratio [AOR] 31.41, 95% CI = 1.16-852.23, P = 0.041), current prednisolone dose ≥ 15 mg/day (AOR 31.22, 95% CI = 1.59-613.85, P = 0.024), serum albumin < 3 mg/dL (AOR 7.97, 95% CI = 1.49-42.56, P = 0.015), and cardiac involvement (AOR = 6.62, 95% CI = 1.08-40.63, P = 0.041) were independent risk factors for SRC. Fifteen SRC patients (78.9%) required dialysis and 10 (52.6%) died.

SRC was an uncommon complication in Thai patients with SSc, but is associated with high mortality. Digital gangrene, current prednisolone dose ≥ 15 mg/day, serum albumin < 3 mg/dL and cardiac involvement were independent risk factors for SRC.