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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
PubMed
Abstract

Insights

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.
Keywords:
CalcinosisSystemic sclerosis

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