AB0805 Clinical features and outcome in scleroderma renal crisis
Authors: S. Monov, D. Monova, E. Miloshova, R. Shumnalieva
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Systemic sclerosis (SSc) is a chronic, autoimmune disease of the connective tissue that involves skin, subcutaneous tissue, muscles and joints, as well as the internal organs: kidneys, lungs, heart. Depending on the extent it can occur as limited (lcSSc) or diffuse (dcSSc) clinical variant. About half of the patients with renal involvement the clinical manifestation is limited to a moderate increase in serum creatinine, mild proteinuria, and moderate hypertension. The most serious complication remains sclerodermal renal crisis (SRC). There was significant heterogeneity in definitions. As a rule for SRC was characterised by new-onset severe hypertension, hypertensive encephalopathy and seizures, acute kidney injury with oliguria, proteinuria and erythrocyturia, and microangiopathic hemolytic anaemia with thrombocytopenia. <h3>Objectives</h3> The aim of our study was to analyse the clinical features and outcome of patients with scleroderma renal crisis. <h3>Methods</h3> We retrospectively reviewed the medical records of patients with scleroderma renal crisis between <sup>January 2002</sup> and December 2016. Conditional logistic regression and multivariate analysis was performed to determine factors independently associated with outcome in patients with SRC. <h3>Results</h3> 18 (14 females and 4 males, median age 47,3±14,6 years) patients diagnosed with SRC were included in the study. The mean duration from first symptom of systemic sclerosis (Raynaud's phenomenon, cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease, pulmonary arterial hypertension, digestive hypomotility and etc.) to SRC attack was 4,47±2,9 years. 13 SRC patients belonged to dcSSc, and 5 patients – to lcSSc. Among SRC patients, 9 were negative of anti-centromere antibodies (ACAs). All these 18 patients had hypertension and renal insufficiency, including 4 dialysis dependent after the onset of SRC and 6 with thrombotic microangiopathy. There were 6 patients receiving renal biopsy (the pathological findings were mainly summarised as intimal thickening and stenosis of renal arterioles). Among 16 patients with long-term followed-up, 15 patients received angiotensin converting enzyme inhibitors (ACEI), 8 patients died, 1 patient was dialysis dependent. Survival from first symptom in those patients with Raynaud's phenomenon mode of onset was higher at 5 years and at 10 years than those with onset as non-Raynaud's phenomenon (p &lt; 0,05). In multivariate analysis, factors related to mortality in SRC were older age at onset, male gender, treatment with corticosteroids, dcSSc subset, interstitial lung disease, pulmonary arterial hypertension, heart involvement, and the mode of onset with non-Raynaud's phenomenon, especially in the form of pulmonary involvement. The mode of onset should be considered an independent prognostic factor in SRS. The treatment of SRC relies on aggressive blood pressure control with an ACEI, combined with other antihypertensive drugs if needed. <h3>Conclusions</h3> SRC usually occurred at the early course of SSc. dcSSc was more frequent than lcSSc. ACAs were found in 50% of SRC patients. The use of ACEIs was still the cornerstone of SRC treatment. <h3>Disclosure of Interest</h3> None declared