Journal Article

AB1218 CALCINOSIS IN SYSTEMIC SCLEROSIS: PREVALENCE, CLINICAL CORRELATIONS, AND AUTOANTIBODY ASSOCIATIONS – A RETROSPECTIVE COHORT STUDY

Pellico, M. R., Sette, M., Cacciapaglia, F., Stano, S., Iannone, C., Minniti, A., Del Papa, N., Caporali, R. F., Armentaro, G., Cavalli, S., Giudice, L., Trignani, G., Angelo, N. D

2024

Overview

Background:Calcinosis, or calcium deposits in skin and soft tissues, is a notable clinical feature of systemic sclerosis (SSc) occurring in around 20% of patients. However, its pathogenesis is unclear and likely involves microvascular injury and dysregulated calcification responses. Calcinosis can lead to painful nodules and ulcers, often on the fingers, and correlates with a history of ischemic digital loss affecting quality of life of patients with SSc. At present, no direct strong association with particular autoantibodies or specific clinical features of the disease has been demonstrated.Objectives:To evaluate prevalence of calcinosis in a Systemic Sclerosis cohort, association with other clinical manifestation and autoantibodies.Methods:A retrospective analysis was conducted on patients that met ACR/EULAR classification criteria for SSc. Clinical characteristics, comorbidities and commonly SSc-associated autoantibodies from these patients were collected. The association between categorical variables was assessed by chi-squared or Fisher exact tests as appropriate; Mann-Whitney test was applied to compare continuous variables between subgroups of patients, as appropriate. Correlation among variables was assessed using Pearson correlation coefficients.Results:The study cohort consisted of 331 SSc patients from our Scleroderma Unit. Of these, 46 (13.8%) presented with at least one calcinosis during the disease course, 27 (58.6%) were classified as limited cutaneous SSc, the other 10 (41,3%) as diffuse cutaneous SSc (dcSSc).Patients with and without calcinosis were compared. Limited cutaneous subtype was equally distributed between the groups. The two groups had comparable median age, age of onset, and female prevalence.Notably, other autoimmune conditions were more common in the calcinosis group (4.3% vs 0.7%, p=0.038). Pitting scars and acral ulcers were also more frequent in patient with calcinosis (54.3% vs 29.8%, p=0.001 and 58.7% vs 33%, p<0.001, respectively).Additionally, gastrointestinal involvement was more prevalent in calcinosis subgroup (56.5% vs 41.4%, p=0.048). In contrast, no significant differences existed between groups in myopathy, neuropathy, sicca symptoms, or autoantibody positivity. Arthralgia was instead less common in the calcinosis group (8.7% vs 22.8%, p=0.027).Positive correlations were confirmed between calcinosis and pitting scars, acral ulcers (coefficients of 0.168, and 0.173 respectively, p < 0.01), other autoimmune diseases, and GI involvement (coefficients of 0.117 and 0.119 respectively, p < 0.05) through Pearson correlation coefficients.Osteoporosis was also more prevalent in patients with calcinosis (43.5% vs 24.9%, p=0.009).Conclusion:The correlations between calcinosis, pitting scars, and acral ulcers highlight the need to better understand the pathophysiological mechanisms linking these manifestations in SSc. One hypothesized mechanism is that vascular injury and ischemia may contribute to dystrophic calcification in affected tissues, as ischemic digital loss is more common in SSc patients with calcinosis.Additionally, the association between calcinosis and osteoporosis suggests a possible connection related to calcium metabolism, though the exact biological mechanisms underlying this relationship remain to be elucidated.Overall, while no strong associations were found between calcinosis and a broader range of clinical features and autoantibodies, this reaffirms the complex, multifactorial nature of calcinosis in SSc. Further research into the pathophysiology, including vascular contributions, is critical to guide targeted treatment development and delineate the clinical implications of this debilitating manifestation for SSc patients.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

• Publisher: BMJ Publishing Group Ltd and European League Against Rheumatism,Elsevier B.V,Elsevier Limited
• Subject: Arthralgia / Autoantibodies / Autoimmune diseases / Bone / Calcification / Calcification (ectopic) / Calcinosis / Calcium metabolism / Comorbidity / Ischemia / Microvasculature / Myopathy / Neuropathy / Osteoporosis / Patients / Quality of life / Scientific Abstracts / Scleroderma / Soft tissues / Systemic sclerosis / Ulcers