responsiveness-to-volume-change-of-calcinosis-cutis-utilizing-dualener

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ISSN 1758-4272 Int. J. Clin. Rheumatol. 2018 133 131-133 131 International Journal of Clinical Rheumatology Case Report Responsiveness to volume change of calcinosis cutis utilizing dual-energy computed tomography in a patient with diffuse cutaneous systemic sclerosis treated with alendronate Rajaie Namas 1 Esat Memisoglu 2 1 Department of Internal Medicine Division of Rheumatology Cleveland Clinic Abu Dhabi Abu Dhabi UAE 2 Department of Radiology Division of Musculoskeletal Imaging Cleveland Clinic Abu Dhabi Abu Dhabi UAE Author for correspondence: namasrclevelandclinicabudhabi.ae Background: A 33-year-old pleasant Emirati lady presented to Cleveland Clinic Abu Dhabi with an established diagnosis of difuse cutaneous systemic sclerosis and calcinosis cutis of 7 years duration from the frst non-Raynaud’s disease manifestation. She was experiencing pain open wounds and visible palpable stones involving the knees. Despite treatment over the years with diferent medications including oral steroids colchicine sodium thiosulphate infusions and mycophenolate mofetil her illness progressed. She was started on alendronate 70 mg weekly on March of 2017 as a treatment option for refractory calcinosis cutis. We examined the usefulness of dual-energy computed tomography DECT in excluding monosodium urate MSU crystal deposition and further quantifed the amount of calcifc deposits to assess responsiveness to alendronate after being on the medication for 1 year. There was a clinically meaningful improvement in knee pain wound healing and volume of calcium deposits pre and post treatment with alendronate. DECT scan is a useful tool that can further characterize the morphology density and distribution of the calcinosis within the soft tissues as well as providing the added beneft of volume quantifcation to assess responsiveness to treatments. Introduction A 33-year-old pleasant Emirati lady presented to the Rheumatology Clinic at Cleveland Clinic Abu Dhabi with an established diagnosis of difuse cutaneous systemic sclerosis dcSSc of 7 years duration from the frst non-Raynaud’s disease manifestation. Since the time of diagnosis she was experiencing progressively worsening painful ulcers associated with soft tissue swelling and recurrent infections involving the knees and was diagnosed with calcinosis cutis. Despite treatment over the years with diferent disease modifying drugs including oral steroids colchicine sodium thiosulphate infusions and mycophenolate mofetil her illness progressed. Physical exam of the knees revealed an erythematous open wound involving the anterior aspect of the knees with visible palpable white stones. Alendronate 70 mg weekly was started on March 2017 and dual- energy computed tomography was utilized to assess the responsiveness to treatment after being on the medication for 1 year. Calcinosis cutis is defned as the deposition of insoluble calcium salt in the skin and subcutaneous soft tissues which is frequently manifested in patients with systemic sclerosis Ssc 12. It occurs in about 25 of patients with SSc and can occur in any subset of SSc but is often seen in limited cutaneous systemic sclerosis 3. Calcium hydroxyapatite is reported to be the major constituent of SSc-associated calcinosis 4. Deposits are commonly found in pressures areas of the elbows knees and hands 5. Patients with calcinosis tend to be older with longer disease duration from initial non-RP symptom 6. Calcium deposits can be palpable on exam and diagnosed by plain radiography 7. Additional types of imaging that can evaluate calcinosis including ultrasound 8 and dual- energy computed tomography 910. We examined the usefulness of dual-energy computed tomography DECT in- • exclusion of monosodium urate MSU crystal deposition and Keywords: calcinosis • systemic sclerosis • dual-energy computed tomography

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132 Namas Memisoglu Case Report Int. J. Clin. Rheumatol. 2018 133 • quantifcation of the amount of calcifc deposits as this can guide in the assessment of treatment response in a patient with symptomatic SSc-associated calcinosis. We quantifed the amount of calcifc deposits prior to starting alendronate 70 mg weekly and a year later to assess treatment response. Tere was clinically meaningful improvement in pain and wound healing images of the wounds not submitted. In addition there was a meaningful decline in the pre and post treatment volume of calcifc deposits in both knees more in the left knee. Figures 1-2 Right knee pre: 21.74 cm 3 post: 18.03 cm 3 Left knee pre: 23.99 cm 3 post: 17.13 cm 3 . Figure 1. Dual energy computed tomography with color rendering obtained in 2017 revealing extensive soft tissue calcium deposits in both anterior knees A axial tomographic and B 3-D volumetric images Figure 2. Dual energy computed tomography with color rendering obtained in 2018 revealing reduction of extensive soft tissue calcium deposits in both anterior knees A axial tomographic and B 3-D volumetric images

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133 Responsiveness to volume change of calcinosis cutis utilizing dual-energy computed tomography Case Report DECT scan can further characterize the morphology density and distribution of the calcinosis within the soft tissues as well as providing the added beneft of volume quantifcation. CT imaging is an option to be considered and utilized in the management of SSc -associated calcinosis which can give more insight in locating these deposits within the soft tissues and can guide in assessing responsiveness to treatments in refractory cases of calcinosis. Acknowledgement I want to thank the patient that give us all the trust to treat her medical condition. References 1. Young A Namas R Dodge C et al. Hand Impairment in Systemic Sclerosis: Various Manifestations and Currently Available Treatment. Curr. Treatm. Opt. Rheumatol. 2 252–269 2016. 2. Valenzuela A Chung L. Calcinosis: pathophysiology and management. Curr. Opin. Rheumatol. 27 542–8 2015. 3. Morrisroe KB Nikpour M Proudman SM. Musculoskeletal Manifestations of Systemic Sclerosis. Rheumatic Diseases Clinics of North America. 413 507–18 2015. 4. Gutierrez A Wetter DA. Calcinosis cutis in autoimmune connective tissue diseases. Dermatol. Ter. 25 195–206 2012. 5. Hsu V Bramwit M Schlesinger N. Use of dual-energy computed tomography for the evaluation of calcinosis in patients with systemic sclerosis. Clin. Rheumatol. 34 1557–61 2015. 6. Chander S Gordon P. Soft tissue and subcutaneous calcifcation in connective tissue diseases. Curr. Opin. Rheumatol. 242 158–64 2012. 7. Chung L Valenzuela A Fiorentino D et al. Validation of a novel radiographic scoring system for calcinosis afecting the hands of patients with systemic sclerosis. Arthritis. Care. Res. 673 425–430 2015. 8. Elhai M Guerini H Bazeli R et al. Ultrasonographic hand features in systemic sclerosis and correlates with clinical biologic and radiographic fndings. Arthritis. Care. Res. 648 1244–1249 2012. 9. Hsu V Bramwit M Schlesinger N. Dual-energy computed tomography for the evaluation of calcinosis in systemic sclerosis. J. Rheumatol. 42 345–6 2015. 10. Freire V Becce F Feydy A et al. MDCT imaging of calcinosis in systemic sclerosis. Clin. Radiol. 683 302– 309 2013.

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