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THU0432 Pericardial effusion is an independent factor predictive of scleroderma renal crisis

Background Scleroderma renal crisis (SRC) adversely affects renal and patient survival in systemic scleroderma (SSc)[.1, 2 The survival rate of SRC has been improved dramatically by angiotensin-converting enzyme inhibitor therapy, but SRC still has a poor prognosis.3 Factors predictive of SRC include early diffuse skin involvement, rapid skin thickening, anti-RNA polymerase (RNAP) III antibodies, arthralgia/synovitis, and high glucocorticoid dosage.4 Although classical data have implicated pericardial effusion as another predictive factor of SRC,5 its role in SRC has not been well established. Objectives To clarify the clinical impact of pericardial effusion as a predictor of SRC. Methods Ninety-five patients diagnosed with SSc at our hospital between January 2003 and December 2017 were enrolled in the study. They were divided into a pericardial effusion group (n=21) and non-pericardial effusion group (n=74), and their clinical features retrospectively compared. Cox-regression analysis was performed to identify factors predictive of SRC. Results The patients comprised 14 men and 81 women with an average age of 57.4 years (range, 14 to 82) and the mean observation period was 65 months (range, 1 to 125). Pericardial effusion was detected in 21 of 95 (22.1%) cases. In the pericardial effusion group, SRC, modified Rodnan’s total skin thickness score (mRSS), C-reactive protein, maximum glucocorticoid dose, proteinuria, finger apex ulcers, and interstitial pneumonia were significantly more prevalent compared to the non-pericardial effusion group. Cox regression analysis indicated that pericardial effusion (hazard ratio; HR 11.8 [95% CI 1.6–84.6], p=0.014) and anti-RNAP III antibodies (HR 11.1 [95% Cl 2.0–59.6], p=0.005) were independent risk factors for SRC, while mRSS (HR 1.0 [95% CI 0.9–1.1], p=0.12), finger apex ulcers (HR 0.57 [95% CI 0.073–4.2], p=0.57), max glucocorticoid dose (HR 1.0 [95% CI 0.9–1.0, p=0.89]), and interstitial pneumonia (HR 0.9 [95%CI 0.2–3.7], p=0.96) were not. In the Kaplan-Meier method, SRC was significantly increased in the pericardial effusion group compared to non-pericardial effusion group (p<0.0001 by log rank test). Conclusions Pericardial effusion is another independent factor predictive of SRC in addition to anti-RNAP III antibodies. References [1] Steen VD, et al. Scleroderma renal crisis. Rheum Dis Clin North Am2003;29:315–33. [2] Hamaguchi Y, et al. Clinical and immunologic predictors of scleroderma renal crisis in Japanese systemic sclerosis patients with anti-RNA polymerase III antibodies. Arthritis Rheumatol2015;67:1045–52. [3] Penn H, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM2007;100:485–94. [4] Woodworth TG, et al. Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Rheumatol2016;12:678–91. [5] Steen VD, et al. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med1984;76:779–86. Disclosure of Interest None declared