Calcinosis in systemic sclerosis: prevalence, clinical picture, management, complications.

This literature review summarizes current data on the epidemiology, pathophysiology, diagnosis and treatment of calcinosis cutis in patients with systemic sclerosis (SSc). The article deals with the analysis of observational studies of the frequency of calcinosis in SSc and associated clinical features, molecular research of potential pathogenetic mechanisms, clinical cases describing new diagnostic approaches and methods for the treatment of calcinosis. Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. Calcinosis is one of the main, poorly managed clinical problems in patients with SSc, affecting at least one quarter of patients with SSc. Calcinosis is associated with a longer disease duration, digital ulcers, acro-osteolysis, positive anticentromere and anti-topoisomerase antibodies. Although the pathogenesis of calcification is not fully known, there is evidence supporting the role of local chronic traumatization, chronic inflammation, vascular hypoxia and malregulation of bone matrix proteins as potential mechanisms for the development of this pathological condition in patients with SSc. The diagnosis can be established on the basis of clinical data or using plain X-ray. Several pharmacological treatment methods have been applied in patients with calcinosis with variable and not so significant results; in turn, surgical excision of calcium deposits remains mainstay of treatment. [ABSTRACT FROM AUTHOR]