Further evidence that calcinosis is associated with repetitive trauma in systemic sclerosis

Hughes, Michael; Hodgson, Richard; Harris, James T.; Porter, Neil; Jackson, Sarah; Kirwadi, Anand; Manning, Joanne; Peytrignet, Sebastien; Herrick, Ariane L.

doi:10.1016/j.semarthrit.2019.07.001

Cited by 11 publications

(9 citation statements)

References 7 publications

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“…Fingertip localization, consistent with the literature, 10,11 may be explained as being the area most frequently exposed to trauma, 19 while we did not notice any different distribution in the two hands or a higher prevalence in one finger compared to the others 20 . In contrast with the literature, 18,21 no difference was identified in the NVC pattern between patients with or without calcinosis, probably because in our cohort the prevalence of a “late” pattern was the most common in both groups, as it has been identified as being associated with DUs by itself 22 …”

Section: Discussionsupporting

confidence: 87%

The impact of skin calcinosis on digital ulcers in patients with SSc: clinical and prognostic stratification using the “wound bed score”

Barsotti

Vicennati

Battista

et al. 2020

International Wound Journal

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Digital ulcers (DUs) represent one of the major burdens for patients with systemic sclerosis (SSc), especially when associated with skin calcinosis (SC). The aim of this work is to evaluate the impact of SC in DUs of patients with SSc for clinical characteristics and prognosis assessed by the wound bed score (WBS). We prospectively enrolled 55 patients with DUs and SSc followed in our dedicated wound care clinic. For all the patients we collected clinical and anthropometric data and characteristics of the DU, and we calculated the WBS for each DU. Ninety‐nine DUs were evaluated (24 with SC). SC was prevalent in limited cutaneous SSc (75%) and in patients with longer disease duration (P = 0.02). SC‐DUs were prevalent at the fingertip (P = 0.04). The healing time was significantly higher in patients with SC (10.4 ± 7.9 weeks) compared with non‐SC (7.0 ± 5.7 weeks) P = 0.03. The WBS negatively correlated with the time to achieve complete healing (r = −0.237 P = 0.023) and the correlation was maintained in the non‐SC (r = −0.46, P = 0.033). DUs in SSc patients with SC are common and difficult to heal. When DUs are treated in dedicated centres, the prognosis is good. The WBS is fast and easy and maybe commonly applied in clinical practice.

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Section: Discussionsupporting

confidence: 87%

The impact of skin calcinosis on digital ulcers in patients with SSc: clinical and prognostic stratification using the “wound bed score”

Barsotti

Vicennati

Battista

et al. 2020

International Wound Journal

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“…Calcinosis in SSc occurs most frequently in the hands, 5,8,39 particularly the fingers, and within the fingers, most commonly in the thumbs or index of the dominant hand. 40,41 Other less common locations include the arms/forearms, knee/proximal lower extremities, feet, and hips. 8,42 These latter sites are thought to be areas affected by trauma or pressure.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…The fact that the index finger and thumb are the two digits most affected by SSc-related calcinosis raises the potential role for repetitive trauma in the pathogenesis of SSc-related calcinosis. 40,41 Similarly, it has been suggested that the release of calcium from mitochondria in muscle cells damaged by myopathy could be a possible mechanism in DM-related calcinosis. 62 Duvvuri et al 63 demonstrated the presence of calcified mitochondria and their remnants in degenerated muscle fibers and blood of JDM patients with calcinosis.…”

Section: Pathogenesismentioning

confidence: 99%

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Valenzuela

Chung

2021

Journal of Scleroderma and Related Disorders

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Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It is a manifestation of several autoimmune connective tissue diseases, most frequently with systemic sclerosis and juvenile dermatomyositis, followed by adult dermatomyositis. Autoimmune connective tissue disease–associated calcinosis is of the dystrophic subtype, which occurs at sites of damaged tissue in the setting of normal serum calcium and phosphate levels. In juvenile dermatomyositis, calcinosis is considered a marker of ongoing disease activity and possibly inadequate treatment, while in adult dermatomyositis, it is a hallmark of skin damage due to chronic rather than active disease. Calcinosis is associated with long disease duration in systemic sclerosis and dermatomyositis, anti-polymyositis/sclerosis autoantibodies in systemic sclerosis and NXP-2 and melanoma differentiation-associated gene 5 in dermatomyositis. Calcinosis in systemic sclerosis occurs most frequently in the hands, particularly the fingers, whereas in dermatomyositis, it affects mainly the trunk and extremities. The primary mineral component of calcinosis is hydroxyapatite in systemic sclerosis and carbonate apatite in dermatomyositis. Calcinosis in dermatomyositis and systemic sclerosis share some pathogenic mechanisms, but vascular hypoxia seems to play a more important role in systemic sclerosis, whereas the release of calcium from mitochondria in muscle cells damaged by myopathy may be a primary mechanism contributing to dermatomyositis-related calcinosis. Multiple treatment strategies for dermatomyositis and systemic sclerosis-related calcinosis have been used with variable results. Early aggressive treatment of underlying myositis in patients with dermatomyositis may improve long-term outcomes of calcinosis. A better understanding of the pathogenesis of calcinosis is needed to improve treatment options.

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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 [2,6,8,20,22,25 ▪▪ ,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%

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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Further evidence that calcinosis is associated with repetitive trauma in systemic sclerosis

Cited by 11 publications

References 7 publications

The impact of skin calcinosis on digital ulcers in patients with SSc: clinical and prognostic stratification using the “wound bed score”

The impact of skin calcinosis on digital ulcers in patients with SSc: clinical and prognostic stratification using the “wound bed score”

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Calcinosis in systemic sclerosis

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