Antonia Valenzuela1, Murray Baron2, Tatiana S Rodriguez-Reyna3, Susanna Proudman4, Dinesh Khanna5, Amber Young5, Monique Hinchcliff6, Virginia Steen7, Jessica Gordon8, Vivien Hsu9, Flavia V Castelino10, Sara Schoenfeld10, Shufeng Li11, Joy Y Wu12, David Fiorentino11, Lorinda Chung13. 1. Pontificia Universidad Católica de Chile, Santiago de Chile, Chile. 2. Division of Rheumatology, Jewish General Hospital, McGill University, Canada. 3. Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubiran, Department of Immunology and Rheumatology, Mexico. 4. Royal Adelaide Hospital North Terrace, Rheumatology Unit and Discipline of Medicine, University of Adelaide, Australia. 5. University of Michigan Scleroderma Program, Department of internal medicine, Division of Rheumatology, USA. 6. Yale University School of Medicine, Section of Rheumatology, Allergy and Clinical Immunology, USA. 7. Georgetown University, Division of Rheumatology, USA. 8. Hospital for Special Surgery, Department of Rheumatology, USA. 9. Rutgers-RWJ Medical School, Rheumatology Division, USA. 10. Harvard Medical School, Division of Rheumatology, USA. 11. Stanford University School of Medicine, Department of Dermatology, USA. 12. Stanford University School of Medicine, Department of Medicine, Division of Endocrinology, USA. 13. Stanford University School of Medicine and Palo Alto VA Health Care System, Department of Immunology and Rheumatology and Dermatology (by courtesy), USA. Electronic address: shauwei@stanford.edu.
Abstract
OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
OBJECTIVE: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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
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