Characteristics of Japanese patients with systemic sclerosis complicated with calcinosis

Takagi, Kae; Kawamoto, Manabu; Higuchi, Tomoaki; Tochimoto, Akiko; Hirose, H; Harigai, Masayoshi; Kawaguchi, Yasushi

doi:10.1111/1756-185x.14121

Cited by 4 publications

(4 citation statements)

References 25 publications

(31 reference statements)

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“…Many studies have established that calcinosis in SSc is associated with features of vascular dysfunction including loss of digital pulp, acro-osteolysis and presence of digital pitting scars [2,6,8,21,23,26 ▪▪ ,27–30]. A Japanese cross-sectional study showed that calcinosis in the facial region is commonly associated with multiple external root resorption which is akin to loss of digital pulp in the hands [19].…”

Section: Associated Disease Characterstics and Pathogenesismentioning

confidence: 99%

“…Based on physical examination or clinically indicated x-rays, it is more common in Caucasians (up to 38%) compared to Asian ethnicity (9%) [2,3]. It has a prevalence of 10% in North Africa [4], 29% in the Middle East, [5] and 24% in Japan [6]. However, subclinical calcinosis detected on imaging studies was found to be present in up to 50% of patients within 4 years of disease onset in Japan [7 ▪ ].…”

Section: Introductionmentioning

confidence: 99%

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Calcinosis in systemic sclerosis

Davuluri,

Lood,

Chung

2023

Current Opinion in Rheumatology

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Purpose of review 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 center 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.

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Section: Associated Disease Characterstics and Pathogenesismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Calcinosis in systemic sclerosis

Davuluri,

Lood,

Chung

2023

Current Opinion in Rheumatology

View full text Add to dashboard Cite

show abstract

“…Many studies have established that calcinosis in SSc is associated with features of vascular dysfunction including loss of digital pulp, acro-osteolysis and presence of digital pitting scars [2,6,8,20,22,25 ▪▪ ,26–29]. A Japanese cross-sectional study showed that calcinosis in the facial region is commonly associated with multiple external root resorption, which is akin to the loss of digital pulp in the hands [30].…”

Section: Associated Disease Characterstics and Pathogenesismentioning

confidence: 99%

“…On the basis of physical examination or clinically indicated x-rays, it is more common in whites (up to 38%) compared with Asian ethnicity (9%) [2,3]. It has a prevalence of 10% in North Africa [4], 29% in the Middle East [5] and 24% in Japan [6]. However, subclinical calcinosis detected on imaging studies was found to be present in up to 50% of patients within 4 years of disease onset in Japan [7 ▪ ].…”

Section: Introductionmentioning

confidence: 99%

Calcinosis in systemic sclerosis

Davuluri

Lood

Chung

2022

Current Opinion in Rheumatology

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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.

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