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Calcinosis in Systemic Sclerosis: Updates in Pathophysiology, Evaluation, and Treatment
Carrie Richardson1,2, Anna Plaas3, John Varga4
1Rush University Medical Center, Chicago, IL, USA. carrie_richardson@rush.edu.
Current Rheumatology Reports
|August 29, 2020
Summary
Systemic sclerosis-related calcinosis involves abnormal mineralization pathways. Future research on phosphate metabolism and PARP enzymes may reveal effective therapies for this common complication.
Area of Science:
- Biomineralization
- Systemic Sclerosis Pathogenesis
- Ectopic Calcification
Background:
- Calcinosis is a frequent complication of systemic sclerosis (SSc).
- Currently, no effective pharmacologic treatments exist for SSc-related calcinosis.
- Patients with SSc-calcinosis exhibit systemic mineralization pathway abnormalities, including reduced inorganic pyrophosphate levels.
Purpose of the Study:
- To review literature on SSc-related calcinosis and other biomineralization disorders.
- To identify potential therapeutic targets for calcinosis.
- To explore the role of phosphate metabolism and PARP enzymes in SSc-calcinosis pathogenesis.
Main Methods:
- Literature review of systemic sclerosis-related calcinosis.
- Analysis of other disorders of biomineralization.
- Investigation of genetic and enzymatic pathways involved in ectopic calcification.
Main Results:
- Systemic abnormalities in mineralization pathways are observed in SSc-related calcinosis.
- Decreased inorganic pyrophosphate levels are noted.
- Phosphate metabolism pathways (involving ABCC6, ENPP1, NT5E genes) and Poly(ADP-ribose) polymerase (PARP) enzymes are implicated in ectopic calcification.
Conclusions:
- Insights from other mineralization disorders highlight the role of phosphate metabolism.
- Knockout models of ABCC6, ENPP1, and NT5E may provide suitable murine models for calcinosis research.
- Further study of phosphate metabolism and PARP enzymes is crucial for understanding SSc-calcinosis pathogenesis and developing treatments.
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