Calcinosis in systemic sclerosis

Abstract

Purpose of reviewThe aim of this study was to provide updated information on the prevalence, pathogenesis, diagnostics and therapeutics of calcinosis cutis associated with systemic sclerosis (SSc).Recent findingsObservational studies show ethnic and geographical differences in the prevalence of calcinosis. In addition to clinical and serological associations, biochemical studies and in-vivo models have attempted to explain theories behind its pathogenesis, including prolonged state of inflammation, mechanical stress, hypoxia and dysregulation in bone and phosphate metabolism. Long-term use of proton pump inhibitors may increase the risk for calcinosis in SSc. Few single-centre observational studies have shown mild benefit with minocycline and topical sodium thiosulfate.SummaryCalcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It affects up to 40% of SSc patients and causes significant morbidity. Long disease duration, features of vascular dysfunction and osteoporosis have been associated with calcinosis. Altered levels of inorganic pyrophosphate and fibroblast growth factor-23 have been implicated in dysregulated phosphate metabolism that may lead to calcinosis in SSc. Plain radiography can help with diagnosis and quantifying the calcinosis burden. Surgical treatment remains the most effective therapy when feasible. At present, no medical therapies have proven efficacy in large randomized controlled trials.

Calcinosis in systemic sclerosis

“…Calcinosis is a term used to describe deposits of insoluble calcium salts in tissues and it is a characteristic imaging finding of systemic sclerosis (SSc) 1,2 . There are currently no effective medical therapies for this progressive, disfiguring, and painful dystrophic calcification 1–3 . Calcinosis can be found in many different tissues, including the superficial soft tissues, periarticular structures, muscles, and tendons but the exact pathogenesis is unknown 1–3 .…”

“…[1][2][3] Calcinosis can be found in many different tissues, including the superficial soft tissues, periarticular structures, muscles, and tendons but the exact pathogenesis is unknown. [1][2][3] Imaging plays a crucial role in the detection of calcinosis and in tracking disease progression. 3 The X-ray is considered a sensitive and specific modality for assessing calcinosis.…”

Calcinosis universalis in systemic sclerosis

“…Calcinosis is a debilitating condition that occurs in 40% of patients with systemic sclerosis. A long disease duration is associated with progressive calcinosis (1). Thus far, there are no effective treatment options for calcinosis.…”

Scrotal Calcinosis in Juvenile Systemic Sclerosis

“…1 Calcinosis in SSc presents as subcutaneous nodules of variable size and shape, most frequently on the fingers, but can also affect other locations. 2 An 18-year-old man with a 10-year history of juvenile SSc presented with multiple scrotal subcutaneous hard lumps. At the time of initial diagnosis, the patient had skin tightness, hand contractures, abnormal nailfold capillaries, and high-titer positive antinuclear antibody with nucleolar pattern.…”