Open AccessInorganic pyrophosphate is reduced in patients with systemic sclerosis
Author(s) -
Vivien Hsu,
Eszter Kozák,
Qiaoli Li,
Márta Bocskai,
Naomi Schlesinger,
Ann K. Rosenthal,
Scott T. McClure,
László Kovács,
Bálint Bálint László,
Szilvia Szamosi,
Gabriella Szücs,
Mary Carns,
Kathleen Aren,
Isaac Goldberg,
András Váradi,
John Varga
Publication year - 2021
Publication title -
rheumatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.957
H-Index - 173
eISSN - 1462-0332
pISSN - 1462-0324
DOI - 10.1093/rheumatology/keab508
Subject(s) - medicine , calcinosis cutis , cohort , calcinosis , rheumatology , gastroenterology , scleroderma (fungus) , pathogenesis , pathology , calcification , inoculation
The pathogenesis of calcinosis cutis, a disabling complication of SSc, is poorly understood and effective treatments are lacking. Inorganic pyrophosphate (PPi) is a key regulator of ectopic mineralization, and its deficiency has been implicated in ectopic mineralization disorders. We therefore sought to test the hypothesis that SSc may be associated with reduced circulating PPi, which might play a pathogenic role in calcinosis cutis.