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Published on: June 16, 2020
Calcinosis in systemic sclerosis.
Srijana Davuluri1, Christian Lood2, Lorinda Chung3
1Stanford School of Medicine, Division of Immunology & Rheumatology, Palo Alto, California.
Calcinosis cutis, a condition affecting up to 40% of systemic sclerosis (SSc) patients, involves calcium deposition in tissues. Research explores its prevalence, causes, and treatments, with surgery being most effective currently.
Area of Science:
- Rheumatology
- Dermatology
- Biochemistry
Background:
- Calcinosis cutis involves insoluble calcium deposition in skin and subcutaneous tissues, impacting up to 40% of systemic sclerosis (SSc) patients.
- Associated factors include long disease duration, vascular dysfunction, and osteoporosis.
- Dysregulated phosphate metabolism, involving inorganic pyrophosphate and FGF-23, is implicated in pathogenesis.
Purpose of the Study:
- To provide updated information on calcinosis cutis in systemic sclerosis (SSc).
- To review prevalence, pathogenesis, diagnostics, and therapeutics.
- To highlight ethnic and geographical variations in calcinosis prevalence.
Main Methods:
- Review of observational studies, biochemical analyses, and in-vivo models.
- Analysis of clinical and serological associations.
- Evaluation of diagnostic methods like plain radiography.
Main Results:
- Prevalence varies geographically and ethnically.
- Potential pathogenetic factors include inflammation, mechanical stress, hypoxia, and altered bone/phosphate metabolism.
- Long-term proton pump inhibitor use may increase risk.
Conclusions:
- Calcinosis cutis causes significant morbidity in SSc patients.
- Surgical intervention is the most effective treatment when feasible.
- No medical therapies have demonstrated proven efficacy in large randomized controlled trials.

