Srijana Davuluri1, Christian Lood2, Lorinda Chung3. 1. Stanford School of Medicine, Division of Immunology & Rheumatology, Palo Alto, California. 2. University of Washington, Division of Rheumatology, Seattle, Washington. 3. Stanford School of Medicine & Palo Alto VA Healthcare System, Division of Immunology & Rheumatology, Palo Alto, California, USA.
Abstract
PURPOSE OF REVIEW: The 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 FINDINGS: Observational 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. SUMMARY: Calcinosis 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.
PURPOSE OF REVIEW: The 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 FINDINGS: Observational 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. SUMMARY: Calcinosis 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.
Authors: Joanne B Manning; Elizabeth Marjanovic; Tonia L Moore; Graham Dinsdale; Sarah Wilkinson; Mark R Dickinson; Ariane L Herrick; Andrea K Murray Journal: Rheumatology (Oxford) Date: 2020-10-01 Impact factor: 7.580
Authors: Vivien M Hsu; Eszter Kozák; Qiaoli Li; Márta Bocskai; Naomi Schlesinger; Ann Rosenthal; Scott T McClure; László Kovács; László Bálint; Szilvia Szamosi; Gabriella Szücs; Mary Carns; Kathleen Aren; Isaac Goldberg; András Váradi; John Varga Journal: Rheumatology (Oxford) Date: 2022-03-02 Impact factor: 7.046