Clinical, biochemical, and radiological characterization of the calcinosis in a cohort of Mexican patients with systemic sclerosis

Abstract

Calcinosis is a frequent complication of systemic sclerosis (SSc) that is usually located in extremities but may occur across the board. The aim of our study was to identify and quantify the distribution of calcinosis in a cohort of Mexican patients with SSc and its association with clinical features and autoantibodies. A cohort of patients with SSc (2013 ACR/EULAR criteria), classified in diffuse cutaneous (dcSSc) and limited cutaneous (lcSSc) (Le Roy criteria), was studied. For their analysis, patients were allocated into those with and without calcinosis (clinical and/or radiological). The evaluation included the modified Rodnan scale for skin and Medsger disease severity scale (DSS). Calcium, phosphorus, vitamin D, and parathyroid hormone (PTH) and antinuclear antibodies and extractable nuclear antigens were determined in serum. A total of 109 patients were included, 41 (37 %) with and 68 (63 %) without calcinosis. Calcinosis was more frequent in patients with dcSSc (55 vs 27 %). In total, we identified 354 sites with calcinosis and mean per patient of 12.0 ± 9.1; the most common sites affected were the hands (83 %), proximal upper extremity (27 %), and proximal lower extremity (22 %). Patients with calcinosis had a higher score of Rodnan scale, Mesdger DSS, and frequency of anti-nucleolar and anti-Scl-70 antibodies compared to those without calcinosis. Abnormal PTH elevation was found in 35 % of patients with calcinosis and 23 % without it. The prevalence of calcinosis is high in Mexican patients with SSc, especially in diffuse variety, and is associated with increased severity of disease.

Calcinosis Cutis Universalis in a Patient with Systemic Sclerosis

“…5 A cohort of Mexican patients with Scl showed the high prevalance of calcinosis, the most common affected sites were the hands (83%), proximal upper and lower extremity (27% and 22% respectively). 6 In our patient with diffuse cutaneous Scl, the symetrical distribution of the sub cutaneous calcinosis was radiologically revealed on the hands, the extremities and the trunk, and they were nonpalpable. The calcinosis can occur superficial, exophytic and lead to ulceration, secondary infection with significant pain.…”

Кальциноз При Системній Склеродермії: Поширеність, Клініка, Менеджмент, Ускладнення

“…SSc-accompanied calcinosis manifests itself with subcutaneous nodes on fingers or in the pressure sites, such as elbows, knees or ischial tuberosities. Calcinosis is most often afflicting hands and feet (65-83 %), proximal upper limbs (27 %), knee joints or proximal lower limbs (10-22%) and thighs (6,7 %) [6]. Calcinosis sites may occur on the trunk, chest, buttocks and other occult places, such as maxillary sinuses, spine and paravertebral tissues [12].…”

“…С.А.Davies et al demonstrate an elevated expression of hypoxia-associated glucose transporter 1 (GLUT1) in the skin biopsy of SSc patients with calcinosis [23]. Several studies [9] also show that presence or history of digital ulcers [11] and/or acroosteolysis [6] predict calcinosis, underlining the role of ischemia. Another study shows vascular endothelial growth factor (VEGF), also known as a potential angiogenic factor induced by hypoxia, when activated, becomes associated with an increased osteoclast activity in SSc patients with acroosteolysis, 73 % of them diagnosed with calcinosis [24].…”

“…This article reviews and summarizes the recent literature data, dealing with calcinosis prevalence, associated clinical factors, pathogenesis, diagnostics and treatment options suggested for dystrophic calcinosis accompanied by SSc. and/or X-ray data, as well as to differences in the patient population [5,6]. This prevalence increases among patients with a long-term SSc history; with calcinosis usually manifesting itself after 10 or more years after diagnosis being established [4,7].…”

“…In the monocentral US study recruiting 215 patients with SSc, calcinosis was associated with a limited SSc form (LF-SSc) [10]; however, in the Mexican and Malaysian cohorts it was associated with a diffuse SSc form (DF-SSc) [6]. Antinuclear and anti-Scl-70 antibodies are more prevalent in the Mexican calcinosis patients than ACAs [6], and antitopoisomerase (Scl-70) antibodies served as calcinosis predictors in the cohort of 1305 SSc patients, according to the Canadian Scleroderma Research Group (CSRG) register, including patients from Canada and Mexico [11]. Furthermore, in the multicentral study of 1009 US Blacks with SSc there were no associations found with skin form, male gender or ACAs [5].…”