AB0606 SYSTEMIC SCLEROSIS – ARE PATIENTS WITH CALCINOSIS DIFFERENT FROM THOSE WHO DO NOT HAVE IT?
Authors: B. Samões, D. Fonseca, R. Vieira, T. Videira, A. Aleixo, P. Pinto, M. Guerra
Journal: Annals of the Rheumatic Diseases (2020)

Background: Systemic Sclerosis (SS) is a heterogenous disease with a broad range of organ involvement. Calcinosis is a common problem and although it may affect almost any body tissue, it is typically seen in the limbs. 1 Its presence relates with higher risk of digital ulcers and infection. 2 It is still unknown whether patients with calcinosis also have other clinical features that differentiate them from the remaining. Objectives: To determine the prevalence of calcinosis in a SS cohort and to evaluate if its presence relates with specific clinical features. Methods: A cross-sectional study was conducted evaluating a cohort of SS patients. Plain radiographs were taken to assess calcinosis at elbows, hands, knees and feet. Clinical data was obtained and analyzed using IBM SPSS Statistics 26®. Results: We included 25 patients, 21 females [n= 21 (84%)], median (min, max) age was 58 (27, 75) years-old. Regarding disease classification, 16 (64%) had limited SS, 4 (16%) had diffuse SS, 3 (12%) had overlap syndrome and 2 (8%) had early SS. Ten (40%) patients had radiological calcinosis in at least one site, seven of which (70%) were subclinical. The most affected areas were knees and hands [n=6 (24%)]. Table 1 summarizes the clinical characteristics of patients with and without calcinosis. Limited SS was significantly more prevalent in the calcinosis group [n=9 (90%) vs. n=7 (46.7%), p=0.04]. All patients had Raynaud phenomenon [n=10 (100%) vs. 15 (100%)]. Current or past digital ulcers [n=5 (50%) vs. n=6 (40%), p=0.697], telangiectasias [n=9 (90%) vs. n=11 (73.3%), p=0.615], pulmonary hypertension [n=2 (20%) vs. n=1 (6.7%), p=0.550] and esophageal involvement [n=6 (60%) vs. n=6 (40%), p=0.428] were more frequent in the calcinosis group but with no statistical significance. Although late capillaroscopic pattern was more frequent in the calcinosis group, there was no statistical significance difference [n=4 (40%) vs. n=1 (6.7%), p=0.121]. Seropositivity for centromere-B antibodies was more frequent in the calcinosis group but with no statistical significance [n=7 (70%) vs. n=8 (53.3%), p=0.678]. Table 1. Demographic and clinical data of patients with and without calcinosis. Demographic and clinical data Calcinosis (n=10 ) No calcinosis (n=15 ) p-value Female gender, n (%) 9 (90) 12 (80) 0.626 Age (years), median [min,max] 68.5 [27, 75] 52 [36, 73] 0.129 Cutaneous classification Limited, n (%) 9 (90) 7 (46.7) 0.04 Diffuse, n (%) 1 (10) 3 (20) 0.626 Early, n (%) 0 (0) 2 (13.3) 0.500 Overlap, n (%) 0 (0) 3 (20) 0.250 Clinical manifestations Current or previous digital ulcers, n (%) 5 (50) 6 (40) 0.697 Interstitial lung disease, n (%) 2 (20) 4 (26.7) 1.000 Pulmonary hypertension, n (%) 2 (20) 1 (6.7) 0.550 Arthritis, n (%) 2 (20) 3 (20) 1.000 Calcinosis, n (%) 3 (30) 0 (0) 0.052 Esophageal involvement, n (%) 6 (60) 6 (40) 0.428 NFC patterns Non specific abnormalities, n (%) 1 (10) 3 (20) 0.626 Early scleroderma, n (%) 1 (10) 1 (6.7) 1.000 Active scleroderma, n (%) 3 (30) 10 (58.8) 0.111 Late scleroderma, n (%) 4 (40) 1 (6.7) 0.121 Autoantibodies Centromere B, n (%) 7 (70) 8 (53.3) 0.678 Scl-70, n (%) 1 (10) 4 (26.7) 0.615 Conclusion: The prevalence of calcinosis was similar to that reported in literature (18-49%). This study confirmed the association, already found in previous studies, between calcinosis and the limited form of SS and raises attention for the importance of calcinosis radiographic screening since there was a high prevalence of subclinical calcinosis. 1 Although there were some clinical differences between patients with and without calcinosis, given the small cohort, statistical significance was not obtained. Larger studies are needed to increase statistical power. References: [1]Valenzuela A et al. Calcinosis in scleroderma. Curr Opin Rheumatol. 2018 Nov;30(6):554-561. [2]Bartoli F et al. Calcinosis in systemic sclerosis: subsets, distribution and complications. Rheumatology (Oxford). 2016 Sep;55(9):1610-4. Disclosure of Interests: None declared