A Case of Refractory Calcinosis in Juvenile Dermatomyositis

Introduction: A 40-year-old woman with Juvenile Dermatomyositis (JDM) diagnosed in childhood experiencing extensive refractory calcinosis universalis. Despite early remission of skin, joint, and muscle disease, severe calcinosis progressed throughout adulthood. Case Description: At age 6, she was seen by a rheumatologist and diagnosed with JDM based on rash, arthralgia, and proximal weakness. She did not have a skin or muscle biopsy, and a myositis panel was unavailable to prognosticate her illness. Initial serology showed positive ANA (>1:2560) and anti-histone antibodies. Anti-Smith, anti-DNA, and anti-NXP2 antibodies (associated with extensive calcinosis) were negative. Her skin, joint, and muscle disease went into remission in her 20s after treatment with methotrexate, mycophenolate mofetil, azathioprine, TNF inhibitors, rituximab, and glucocorticoids. However, calcinosis continued to worsen, ulcerate, and become infected. Treatment with sodium thiosulfate, colchicine, simvastatin, and calcium-channel blockers (CCB) was attempted but refractory. Chronic infections, immune dysfunction, and kidney disease limited the use of immunosuppression and further augmented dystrophic calcification. Discussion: Calcinosis is a well-recognized feature of many connective tissue diseases. Although common in JDM, it is less frequent in the adult form, affecting about 10% of patients. Calcinosis can be classified into subtypes; dystrophic calcification is the most common and occurs with normal calcium and phosphate levels. Treatment is based on case series, with colchicine, sodium thiosulfate, probenecid, CCBs, bisphosphonates, and TNF inhibitors showing variable results. Early aggressive treatment of disease activity remains the standard for prevention. This case highlights the need for further study of refractory calcinosis and current therapeutic limitations.Multiple bulky cutaneous and muscular calcifications throughout the chest wall.