Background Calcification of the skin and subcutaneous tissue is known to occur in a variety of disorders and may be classified as dystrophic, metastatic, idiopathic or iatrogenic calcification, and calciphylaxis. It occurs in several connective tissue diseases, mainly systemic sclerosis and dermatomyositis, and can cause immense discomfort, pain and/or ulceration. The pathophysiology of the disorder is still unclear. Several treatment modalities have been tried to relieve this condition without any effect.
Calciphylaxis is a condition that occurs in patients with end-stage renal disease. Successful treatment of calciphylaxis with sodium thiosulfate has been reported (1, 2). Sodium thiosulfate is a potent antioxidant and vasodilator that also chelates and dissolves calcium deposits. Several case reports of successful treatment with a combination of abatacept and sodium thiosulfate in juvenile dermatomyositis with severe calcinosis have been published (3, 4).
Objectives We treated four patients, two with dermatomyositis and two with systemic sclerosis, all patients had progressive calcinosis. All four patients were women with a mean age of 64 years (56-72), disease duration of 13.5 years (11-18). The duration of calcinosis cutis was from 6 to 13 years. Several attempts had been made to halt the progression of calcinosis in these four patients, including therapy with diltiazem, risendronate and immunosuppressive agents. We chose the treatment protocol as described in the above mentioned case reports. Treatment with abatacept 10mg/kg, (administration at 0, 2, and 4 weeks and then monthly thereafter) was initiated. In addition, the patients received 10 g IV sodium thiosulfate 3 times weekly for 2 weeks, then 15g 2 times weekly for the next 3 months. For objective evaluation of calcinosis the plain X-rays were performed before treatment start and 3 months thereafter.
Results We observed no change of the size of calcinosis in any of the patients following 3 months of combined abatacept and sodium thiosulfate treatment. We observed no serious side-effects of the treatment.
Conclusions We report the use of abatacept and sodium thiosulfate in four cases of calcinosis cutis associated with connective tissue disease. We did not achieve any reduction of calcinosis. However, no increase of the calcinosis was observed either. The combination therapy resulted in halting the progression of calcinosis during the treatment period. Possibly, this treatment modality could be more effective if started earlier in the disease.
Garcia CP, Roson E, Peon G, Abalde MT, De La Torre C. Calciphylaxis treated with sodium thiosulfate: report of two cases. Dermatol Online J. 2013 Sep 14;19(9):19616.
Ning MS, Dahir KM, Castellanos EH, McGirt LY. Sodium thiosulfate in the treatment of non-uremic calciphylaxis. J Dermatol. 2013 Aug;40(8):649-52. doi: 10.1111/1346-8138.12139. Epub 2013 May 10.
Arabshahi B, Silverman RA, Jones OY, Rider LG. Abatacept and sodium thiosulfate for treatment of recalcitrant juvenile dermatomyositis complicated by ulceration and calcinosis. J Pediatr. 2012 Mar;160(3):520-2. doi: 10.1016/j.jpeds.2011.11.057. Epub 2012 Jan 13
Pagnini I, Simonini G, Giani T, Marrani E, Moretti D, Vannucci G, Cantarini L, Cimaz R. Sodium thiosulfate for the treatment of calcinosis secondary to juvenile dermatomyositis.Clin Exp Rheumatol. 2013 Nov 14.
Disclosure of Interest None declared