Systemic sclerosis skin is a primed microenvironment for soft tissue calcification—a hypothesis

Burgess, Kyle A.; Herrick, Ariane L.; Watson, Rachel

doi:10.1093/rheumatology/keab156

Cited by 9 publications

(9 citation statements)

References 82 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It is likely that bone resorption, as it occurs in acro-osteolysis and osteoporosis, releases inorganic phosphate, which promotes osteoblastic differentiation of dermal fibroblasts [1]. Consistent with this interpretation, MSCs underwent osteogenic transformation upon in-vitro exposure to appropriate osteogenic media consisting of beta-glycerophosphate [41].…”

Section: Associated Disease Characterstics and Pathogenesismentioning

confidence: 75%

“…Several genes involved in maintaining PPi levels, including ABCC6 (ATP binding cassette), ENPP1 (ectonucleotide pyrophosphatase phosphodiesterase 1) and NT5E (ecto- 5’ nucleotidase), have been implicated in other ectopic mineralization disorders [14,42]. The ENPP1 pathway may also be inhibited by inflammatory cytokines, in particular IL-1β [41]. PPi levels are reduced in ectopic mineralization disorders such as pseudoxanthoma elasticum.…”

Section: Associated Disease Characterstics and Pathogenesismentioning

confidence: 99%

See 1 more Smart Citation

Calcinosis in systemic sclerosis

Davuluri

Lood

Chung

2022

Current Opinion in Rheumatology

View full text Add to dashboard Cite

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.

show abstract

Section: Associated Disease Characterstics and Pathogenesismentioning

confidence: 75%

Section: Associated Disease Characterstics and Pathogenesismentioning

confidence: 99%

Calcinosis in systemic sclerosis

Davuluri

Lood

Chung

2022

Current Opinion in Rheumatology

View full text Add to dashboard Cite

show abstract

“…Ultrasonography is mostly used to assess the level of blood clots in patients. [126][127][128][129][130] Abbreviations: Anti-NXP-2 antibody, anti-nuclear matrix protein 2 antibody; APS, antiphospholipid syndrome; DcSSc, diffuse cutaneous SSc; DM, dermatomyositis; FDG-PET, Fluorodeoxyglucose positron emission tomography; GCA, giant cell arteritis; JDM, juvenile dermatomyositis; lcSSc, limited cutaneous SSc; PsA, psoriatic arthritis; RA, rheumatoid arthritis; SLE, systemic lupus erythematosus; SSc, systemic sclerosis.…”

Section: Differential Diagnosismentioning

confidence: 99%

Calciphylaxis and its co‐occurrence with connective tissue diseases

Zhou

Chen

Yin

et al. 2022

International Wound Journal

View full text Add to dashboard Cite

Calciphylaxis, also known as calcific uremic arteriopathy, is a rare calcification syndrome that presents as ischemic skin necrosis and severe pain. It has a high mortality rate and is characterised by calcification of the small and medium arteries and micro‐thrombosis. Calciphylaxis mainly occurs in patients with end‐stage renal disease. In recent years, there have been an increasing number of cases of calciphylaxis associated with connective tissue diseases. Given the absence of clear diagnostic criteria for calciphylaxis thus far, an early diagnosis is crucial for designing an effective multidisciplinary treatment plan. In this article, we review the research progress on calciphylaxis and describe its characteristics in the context of connective tissue diseases.

show abstract

“…Calcinosis is a form of ectopic calcification and biomineralization in which the main mineral component is predominantly hydroxyapatite and is covered by a cell-free connective tissue matrix ( Burgess et al, 2021 ). No definitive treatment method for calcinosis has been established.…”

Section: Introductionmentioning

confidence: 99%

Cancellous bone-like tissue replacement from calcinosis in patients with systemic sclerosis with multiple external root resorption

et al. 2022

View full text Add to dashboard Cite

Calcinosis is frequently observed in patients with systemic sclerosis (SSc). The fundamental treatment of calcinosis has not yet been established. During follow-up, calcinosis in the subcutaneous surface is often spontaneously extracted or remains confined by fibrous tissues. We previously identified a new symptom in SSc patients, multiple external root resorption (MERR), and these patients had calcifications in the nasal spine. Here, we report for the first time that calcinosis at the nasal spine in patients with MERR can be replaced by cancellous bone-like tissue. Patients 1 and 2 were a 62-year-old Japanese female and a 45-year-old Japanese female (respectively) with MERR who had been previously treated for SSc (Patient 1: limited type, positive for anti-centromere antibody; Patient 2: diffuse type, positive for anti-Scl70 and anti-SS-A antibodies). Patient 3 was a 57-year-old female with MERR who had been previously treated for SSc (diffuse type, positive anti-Scl-70 antibody) and underwent denosumab injection for osteoporosis. Cone-beam computed tomography (CBCT) and CT images in the calcifications at the nasal spine in Patient 1 and 2 were replaced with cancellous bone-like tissue, but not in Patient 3. Serum laboratory examination was performed to assess the systemic bone disease. All three patients had normal clinical data within the references, apart from slightly higher 1,25-dihydroxyvitamin D levels in Patient 1. SSc patients with calcinosis in the maxillofacial area need to be examined carefully for bone replacement using CBCT or CT.

show abstract

Systemic sclerosis skin is a primed microenvironment for soft tissue calcification—a hypothesis

Cited by 9 publications

References 82 publications

Calcinosis in systemic sclerosis

Calcinosis in systemic sclerosis

Calciphylaxis and its co‐occurrence with connective tissue diseases

Cancellous bone-like tissue replacement from calcinosis in patients with systemic sclerosis with multiple external root resorption

Contact Info

Product

Resources

About