| Abstract [eng] |
Childhood idiopathic inflammatory myopathies are rare, serious chronic conditions of which the most common is juvenile dermatomyositis(1). Calcinosis is one of the most severe and life-threatening complications of juvenile dermatomyositis. It affects approximately 10-70% of patients and leads to functional disability and low quality of life. Calcinosis in juvenile dermatomyositis is associated with delayed diagnosis and treatment, inadequate initial treatment, and prolonged disease activity. Therefore, prompt diagnosis, quick initiation of induction therapy with corticosteroids, and early aggressive escalation of treatment to control disease activity are essential to prevent the development of calcinosis(3). Currently, different therapies are used to treat calcinosis. Due to the rarity of the main disease and its rare complications, these different treatment methods are based on case studies or individual experiences of institutions. The main therapies are drugs that affect the metabolism of calcium and phosphorus, and biological therapy. However, their use to treat calcinosis was associated with controversial therapeutic effects(4). There have been no evidence-based studies to develop an international consensus on the treatment of calcinosis in juvenile dermatomyositis. In this paper, two clinical cases of patients with juvenile dermatomyositis who have developed progressive calcinosis in the course of the disease are described. A 7-year-old boy and a 9-year-old girl had a typical clinical manifestation of juvenile dermatomyositis. Although patients were diagnosed early, aggressive treatment was given in time, and extractable nuclear antigen antibodies with poor prognosis were not detected – without adequate control of the disease activity calcinosis was developed. The use of diltiazem showed a positive effect in controlling the progression of calcinosis. However, the effect was insufficient in the reported cases. Other therapies for the treatment of calcinosis are considered on an individual basis. Recent literature on juvenile dermatomyositis was reviewed and the options of treatment for progressive calcinosis, and the use of Janus kinase inhibitors were evaluated. |
