1063: Assessment of Calcinosis in Portuguese Patients with Systemic Sclerosis – a Multicenter Study

Beatriz Samões¹, Diogo Fonseca¹, Tiago Beirão¹, Flavio Costa¹, Romana Vieira¹, Georgina Terroso², Raquel Ferreira², Rafaela teixeira³, André Saraiva⁴, Maria João Henriques⁵, Ana Catarina Duarte⁶, Ana Cristina Cordeiro⁶, Paulo Vilas-Boas⁷, Inês Genrinho³, Ana Bento da Silva⁸, Laura Gago⁸, Catarina Resende⁹, Patricia Martins⁹, Nathalie Madeira¹⁰, Sara Paiva Dinis¹⁰, Maura Couto³, Inês Santos³, Filipe Araújo¹¹, Ana Filipa Mourão¹¹, Miguel Guerra¹², Margarida Oliveira¹², Alexandra Daniel¹³, Marília Rodrigues¹³, Catarina Soares¹⁴, Hugo Parente¹⁵, Carolina Furtado¹⁶, Tomás Fontes¹⁶ and Joana Abelha-Aleixo¹, ¹Rheumatology Department, Centro Hospitalar de Vila Nova de Gaia / Espinho, Vila Nova de Gaia, Portugal, ²Rheumatology department, Centro Hospitalar Universitário de São João, Porto, Portugal, ³Rheumatology department, Centro Hospitalar Tondela-Viseu, Viseu, Portugal, ⁴Rheumatology department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal, ⁵Rheumatology department, Centro Hospitalar e Universitário de Coimbra, Lisboa, Portugal, ⁶Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, ⁷Rheumatology Department, Centro Hospitalar Baixo Vouga, Aveiro, Portugal, ⁸Rheumatology department, Hospital de Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal, ⁹Rheumatology department, Centro Hospitalar e Universitário de Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisboa, Portugal, ¹⁰Rheumatology department, Unidade Local de Saúde da Guarda - Hospital Sousa Martins, Guarda, Portugal, ¹¹Rheumatology and Osteoporosis Unit, Hospital de Sant´Ana, SCML, Lisboa, Portugal, ¹²Rheumatology Department, Centro Hospitalar Universitário da Cova da Beira, Covilhã, Portugal, ¹³Rheumatology department, Hospital Distrital de Leiria, Leiria, Portugal, ¹⁴Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Viana do Castelo, Portugal, ¹⁵Rheumatology department, Unidade Local de Saúde do Alto Minho, Ponte de Lima, Portugal, ¹⁶Rheumatology department, Hospital do Divino Espírito Santo, Ponta Delgada, Portugal

Background/Purpose: Calcinosis is a challenging problem among Systemic Sclerosis (SSc) patients with a reported prevalence of 18-49%. We aim to define the prevalence of clinical and subclinical calcinosis among Portuguese SSc patients, currently unknown, as well as the most sensitive anatomical location and the sensitivity of the clinical method for its diagnosis. We also aim to clarify the phenotype of SSc patients with calcinosis.

Methods: A cross-sectional multicenter study was conducted evaluating SSc patients from 14 Portuguese centers, registered in the Rheumatic Diseases Portuguese Registry (Reuma.pt), that fulfilled Leroy/Medsger 2001 or ACR/EULAR classification criteria for SSc. The assessment of calcinosis was made systematically through clinical examination and plain radiographs of hands, elbows, knees and feet, analyzed by 2 rheumatologists of each center. Statistical analysis with independent parametric or non-parametric tests, multivariate logistic regression of the significant variables in univariate analysis and sensitivity calculation of each radiographed site and of the clinical method for the diagnosis of calcinosis were performed.

Results: 226 patients were included, of whom 191 were female (84.5%), with a median [min, max] age of 64 [20, 90] years-old and a median [min, max] disease duration of 11.47 [1, 62] years. 172 patients (76.4%) had limited SSc, 36 (16%) diffuse SSc, 9 (4%) sine scleroderma SSc and 8 (3.6%) early SSc. Clinical calcinosis was described in 63 (28.1%) patients, 10 of which was not radiologically confirmed. 91 (40.3%) patients had radiological calcinosis in at least one site [hand in 68 (74.7%), knee in 32 (35.2%), elbow in 30 (34.1%) and foot in 21 (23.9%)], of which 37 (40.7%) were subclinical. The most sensitive location to detect calcinosis was the hand (74.7%) while the sensitivity of the clinical method was 58.2%. Table 1 summarizes the clinical characteristics of patients with and without calcinosis. Patients with calcinosis were more often female (p=0.008), older (p < 0.001) and with longer disease duration (p < 0.001). The presence of calcinosis was significantly associated with limited SSc (p=0.017), telangiectasia (p=0.039), digital ulcers (p=0.001), oesophageal (p < 0.001) and intestinal (p=0.003) involvements. Osteoporosis and late scleroderma capillaroscopic pattern (p < 0.001) were significantly more often on calcinosis group (p=0.028). In multivariate analysis, digital ulcers (OR 2.631, 95%CI 1.022-6.775, p=0.045) were found to be predictors of overall calcinosis, oesophageal involvement (OR 3.521, 95%CI 1.282-9.668, p=0.015) and osteoporosis (OR 4.083, 95%CI 1.176-14.176, p=0.027) of hand calcinosis and late capillaroscopic pattern (OR 7.608, 95%CI 1.661-34.858, p=0.009) of knee calcinosis.

Conclusion: The prevalence of overall calcinosis was in the range of that previously reported. The higher prevalence of subclinical calcinosis suggests that calcinosis might be underdiagnosed and that radiographic screening in SSc patients might be relevant. The differences between patients with and without calcinosis are in agreement with the literature. Calcinosis predictors appear to vary according to location and suggest a multifactorial pathogenesis.
Table 1 - Demographic and clinical features of SSc patients with and without calcinosis.
Disclosures: B. Samões, None; D. Fonseca, None; T. Beirão, None; F. Costa, None; R. Vieira, None; G. Terroso, None; R. Ferreira, None; R. teixeira, None; A. Saraiva, None; M. Henriques, None; A. Duarte, None; A. Cordeiro, None; P. Vilas-Boas, None; I. Genrinho, None; A. Bento da Silva, None; L. Gago, None; C. Resende, None; P. Martins, None; N. Madeira, None; S. Paiva Dinis, None; M. Couto, None; I. Santos, None; F. Araújo, None; A. Mourão, None; M. Guerra, None; M. Oliveira, None; A. Daniel, None; M. Rodrigues, None; C. Soares, None; H. Parente, None; C. Furtado, None; T. Fontes, None; J. Abelha-Aleixo, None.