Management of Calcinosis Associated with Dermatomyositis

Abstract

Calcinosis cutis, or dystrophic soft-tissue calcification, is a common and debilitating complication of adult and juvenile dermatomyositis. Dermatomyositis-associated calcinosis is difficult to treat and is associated with significant morbidity. The purpose of this review is to provide an update of treatment modalities for calcinosis in dermatomyositis based on published studies. Specific guidelines are lacking for calcinosis cutis management. Based on previous case reports, case series, cohort studies, and limited controlled studies, medications including diltiazem, bisphosphonates, sodium thiosulfate, aluminum hydroxide, warfarin, probenecid, colchicine, minocycline, and intravenous immunoglobulins have been used to control calcinosis progression in dermatomyositis, but no treatment has convincingly prevented or reduced calcinosis. Surgical excision of large or symptomatic calcium deposits remains the mainstay of treatment. Biologic therapies including infliximab, abatacept, rituximab, anakinra, and the oral JAK inhibitor tofacitinib have been used to control dermatomyositis-associated calcinosis in case reports and series. Pharmacological treatment aimed to reduce calcinosis is challenging given that no therapies have consistently been found to be effective and few studies have a high level of evidence. Randomized controlled trials using novel outcome measures are necessary to evaluate the efficacy of proposed and emerging therapies. Trial registration: clinicaltrials.gov NCT03639779 and NCT03267277