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Calcinosis in systemic sclerosis: An update on pathogenesis, related complications, and management: A heavy burden
Aslihan Avanoglu-Guler1, Corrado Campochiaro2, Giacomo De Luca2
1Department of Internal Medicine, Division of Rheumatology, Gazi University, Ankara, Turkey; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy; Vita-Salute San Raffaele University, 20132 Milan, Italy.
Abstract:
In SSc, dystrophic calcinosis is one of the major clinical manifestations, characterized by the deposition of insoluble calcific substances in tissues, predominantly in the chemical form of calcium hydroxyapatite. Furthermore, calcinosis might lead to compressive neuropathies and severe pain. Current evidence suggests that tissue ischemia and repeated trauma are implicated in the development of calcinosis; however, there are still too many unknown areas that need to be investigated. Detection of calcinosis is commonly performed using X-ray or ultrasound. Moreover, quantification of calcinosis with X-ray and dual-energy computed tomography might be useful for the assessment of disease burden and monitoring of the disease. Despite its prevalence and clinical outcomes, there are no approved disease-modifying treatments for calcinosis in SSc. Debulking or surgical intervention might be preferred for calcinosis complicated with infection, compressive symptoms, or relief of pain. Therefore, innovative investigations and tailored therapeutic approaches are urgently needed to lift the burden of calcinosis from the hands of SSc patients.
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