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Calcinosis is associated with ischemic manifestations and increased disability in patients with systemic sclerosis

2020; Elsevier BV; Volume: 50; Issue: 5 Linguagem: Inglês

10.1016/j.semarthrit.2020.06.007

1532-866X

Antonia Valenzuela, Murray Baron, Tatiana Sofia Rodrı́guez-Reyna, Susanna Proudman, Dinesh Khanna, Amber Young, Monique Hinchcliff, Virginia Steen, Jessica Gordon, Vivien Hsu, Flavia V. Castelino, Sara Schoenfeld, Shufeng Li, Joy Y. Wu, David Fiorentino, Leland W.K. Chung,

Hypertrophic osteoarthropathy and related conditions

Calcinosis is a debilitating complication of systemic sclerosis (SSc) with no effective treatments. We sought to identify clinical correlations and to characterize complications and disability associated with calcinosis in a multi-center, international cohort of SSc patients.We established a cohort of 568 consecutive SSc patients who fulfill 2013 revised ACR/EULAR criteria at 10 centers within North America, Australia, and Mexico. Calcinosis was defined as subcutaneous calcium deposition by imaging and/or physical examination, or a clear history of extruded calcium. All patients completed the Scleroderma Health Assessment Questionnaire Disability Index and Cochin Hand Functional Scale.215 (38%) patients had calcinosis. In multivariable analysis, disease duration (OR=1.24, p = 0.029), digital ischemia (OR=1.8, p = 0.002) and Acro-osteolysis (OR=2.97, p = 0.008) were significantly associated with calcinosis. In the subset of patients with bone densitometry (n = 68), patients with calcinosis had significantly lower median T-scores than patients without (-2.2 vs. -1.7, p = 0.004). The most common location of calcinosis lesions was the hands (70%), particularly the thumbs (19%) with decreasing frequency moving to the fifth fingers (8%). The most common complications were tenderness (29% of patients) and spontaneous extrusion of calcinosis through the skin (20%), while infection was rare (2%). Disability and hand function were worse in patients with calcinosis, particularly if locations in addition to the fingers/thumbs were involved.We confirmed a strong association between calcinosis and digital ischemia. Calcinosis in SSc patients most commonly affects the hands and is associated with a high burden of disability and hand dysfunction.