Issue 8/2026
Vasilev, T.
Clinic of Rheumatology, Children’s Hospital “Ivan Mitev” – Sofia
Department of Pediatrics; Medical University – Sofia
Juvenile dermatomyositis is a rare and heterogeneous immune-mediated inflammatory myopathy characterized by muscular, cutaneous, and variably severe systemic involvement. Early initiation of combined treatment with systemic glucocorticoids and methotrexate remains the cornerstone of management; however, some patients develop chronic active or refractory disease and accumulate permanent damage. The contemporary treat-to-target strategy defines inactive disease as the preferred therapeutic goal and introduces intermediate time points for assessing treatment response and adapting therapy promptly. Advances in the understanding of interferon signaling, B- and T-cell activation, and other immune mechanisms provide a pathogenetic rationale for targeted therapies. Myositis-specific autoantibodies support the identification of distinct clinical phenotypes and risk stratification, whereas the interferon gene signature, CXCL10, galectin-9, and monocyte SIGLEC-1 are being investigated as dynamic biomarkers of disease activity. Rituximab has the most extensive accumulated clinical experience among biological therapies, while findings for abatacept and Janus kinase inhibitors, particularly tofacitinib, are encouraging in refractory disease. Nevertheless, the current evidence is derived mainly from small prospective studies, retrospective cohorts, and case series. Targeted therapies therefore do not replace standard initial treatment but represent an individualized option for carefully selected patients.
Key words: juvenile dermatomyositis; biomarkers; targeted therapy
Address for correspondence:
Teodor Vasilev, MD, PhD
Clinic of Rheumatology,
Children’s Hospital “Ivan Mitev” – Sofia
11, Аcad. Ivan Geshov, Blvd.
1612, Sofia
e-mail: tvasilev@medfac.mu-sofia.bg