AB0781 Glucocorticoid dose and cardiac involvement might be potential risk factors for scleroderma renal crisis
Authors: X.-N. Wei, D.-H. Zheng, J.-J. Liang, L. Dai
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc). SRC remains a major risk factor for mortality in SSc. It is important to identify potential risk factors for SRC, and avoid developing overt SRC. <h3>Objectives</h3> To perform a retrospective case series analysis of the characteristics, management and outcomes of SRC in Chinese SSc patients. <h3>Methods</h3> SSc patients hospitalised at Sun Yat-Sen Memorial Hospital from January 1992 to December 2017 were recruited. Clinical data were collected. SRC was defined as new onset, with blood pressure (BP) &gt;140/90 mmHg or a&gt;30 mmHg rise in BP from baseline, rising serum creatinine (Scr) levels and/or oligoanuria. Data were showed as mean ±standard deviation. <h3>Results</h3> (<sup>1</sup> There were 749 SSc patients recruited and 16 patients (2.1%) of them were hospitalised for SRC. Among these 16 patients, 56% were females, age was 54.6±13.6 years, mean duration from SSc onset to SRC occurred was 4 years. (<sup>2</sup> SRC developed in 14 patients (87.5%) with diffuse cutaneous SSc (dcSSc), and in 2 patients (12.5%) with limited cutaneous SSc (lcSSc). Eleven patients (68.8%) were under glucocorticoid treatment before SRC onset: 4 patients received ≥30 mg/d of prednisone, 6 patients received ≥7.5 mg/d prednisone and 1 patient received &lt;7.5 mg/d prednisone. No patient was treated with angiotensin-converting enzyme (ACE) inhibitors before SRC. (<sup>3</sup> All 16 patients manifested progressive renal failure, with Scr levels increase to 420±256 μmol/L. Ten patients manifested new onset hypertension, with systolic BP 175±21 mmHg and diastolic BP108 ±13 mmHg. Five patients who had a history of well-controlled hypertension manifested accelerated increase in BP 178±17/108±7 mmHg. One patient was normotensive, but manifested rapidly progressive oliguric renal failure with Scr increase to 969 μmol/L, massive proteinuria and hemolytic anaemia. (<sup>4</sup> Twelve patients (75%) had pulmonary fibrosis, 11 patients (68.8%) had cardiac involvement, 6 patients had pulmonary arterial hypertension (PAH) and 6 patients had gastrointestinal dysfunction. Cardiac involvement was common, manifested pericarditis, myocardial damage and heart failure (n=7, 43.8%, respectively). All 5 dead patients were accompanied by cardiac involvement. (<sup>5</sup> Eleven patients had Raynaud's phenomenon, 8 patients had digital ulcers, 5 patients had arthritis and 2 patients had oliguria. Thirteen patients (81%) manifested anaemia, 8 patients (50%) manifested thrombocytopenia, and 8 patients (50%) manifested microangiopathic haemolytic anaemia (MAHA). Eleven patients (68.8%) received ACE inhibitor treatment. Fifteen patients were treated with glucocorticoid and 12 patients with immunosuppressant (Cyclophosphamide n=10, Azathioprine n=2). After treatment, renal recovered in 4 patients (25%), kidney function improved and developed to chronic kidney disease (CKD) without dialysis in 5 patients (31%), 2 patients required permanent dialysis (13%). Five patients (31%) died. <h3>Conclusions</h3> Medium-to-high doses of glucocorticoids (prednisone ≥7.5 mg/d) were associated with an increased risk of SRC. Cardiac involvement was common and associated with high mortality in SRC patients. <h3>Disclosure of Interest</h3> None declared