AB0690 Calcinosis cutis in systemic sclerosis: clinical and biological associations

Abstract

BackgroundCalcinosis cutis (CC) is a rare disorder characterized by the abnormal deposition of calcium salts in the subcutaneous tissues of patients with normal calcium metabolism. Dystrophic calcinosis is a common and potentially debilitating manifestation in systemic sclerosis (SSc) patients. The CC was described to appear more frequently in newly diagnosed SSc patients with Raynaud’s phenomenon, with ischemic ulcer or telangiectasia and in areas related to repeated trauma, but current data remains inconclusive 1,2.ObjectivesOur objective was to analyze the prevalence, clinical features, and biological associations of CC in SSc patients.MethodsA retrospective analysis of patients hospitalized in the Rheumatology Department of the Colentina Clinical Hospital, Bucharest, Romania. Data of the patients fulfilling the 2013 American College of Rheumatology/ European League Against Rheumatism (ACR/EULAR) SSc classification criteria were collected. In all patients, clinical examination, including evaluation of the cutaneous involvement by the modified Rodnan skin score (mRSS) was performed. A CC diagnosis was sustained by radiographic and/ or ultrasound imaging.Results44 SSc patients were included with female predominance (39 patients - 88.6%), and with mean (SD) age 58.8±11.3 years old, CC was found in 11(25.0%) of these patients, all feminine gender. Elbow (36.4%), hands and forearms (27.3%) were the most frequent sites affected by CC.Overall, CC was not related to the SSc subtype, when comparing limited (lc) with diffuse cutaneous (dc) involvement (27.3% vs. 48.5%, p=0.219). However, patients with CC had more often lc SSc, 72.7% vs. 27.3%. Accordingly, SSc patients with CC had a significant lower mRSS score, 6 (1;7) vs. 9 (5; 15.5) points, p=0.001 and more frequently expressed positives anti-centromere antibodies (ACA), 72.7% vs. 57.1%, p=0.036. CC was also found not to be related to sclerodactyly, 54.5% vs. 75.8%, p=0.038. Regarding the nailfold capillaroscopy (NFC) exam, the CC was more often described in patients with late NFC pattern, 45.5% of cases.In SSc patients with CC, there was reported more often intestinal transit disturbances 45.5% vs. 3.0%, p=0.015, even if overall there was no gastrointestinal involvement, 72.7% vs. 63.6%, p=0.582.Regarding inflammation, the level of erythrocyte sedimentation rate (ESR) was lower in patients with CC 18 (13;33) vs. 33 (17.0; 58.5) mm/h, p=0.040, but not the C-reactive protein level 2.4 (1.7; 6.7) vs. 3.3 (1.2; 8.5) mg/L, p=0.866.When comparing SSc patients with or without CC we did not found any significant difference regarding the presence of anti-Scl70 antibodies, p=0.432, rheumatoid factor, p=0.067, lupus anticoagulant or antiphospholipid antibodies, p=0.835. Also, no significant correlations were identified for CC in SSc and the following parameters: smoking, p=0.162, obesity, p=0.812, hypertension, p=0.371, diabetes, p=0.403, Raynaud phenomenon, p=0.539, telangiectasia, p=0.28, pulmonary hypertension, p=0.777, lung interstitial disease, p=0.315, digital ulcers, p=0.723, or digital starry scars, p=0.223.ConclusionThe CC prevalence in SSc consecutive patients was 25.0%, more frequently with elbow localization. We’ve also found more frequent CC in patients with positive ACA serology and defined relations with many other SSc-related parameters.