Dermatomyositis Management Requires Risk-Based Screening for Systemic Complications

Recognizing distinct dermatomyositis (DM) phenotypes and assessing patients for systemic complications can guide evaluation and treatment, according to Joseph F. Merola, MD, MMSc, FAAD, FACR.

During his session, “Recognizing and Managing Dermatomyositis: From Rash to Systemic Disease,” presented at the Masterclasses in Dermatology NP/PA Institute, Dr Merola reviewed myositis-specific autoantibodies, risk-based screening for interstitial lung disease (ILD) and malignancy, and current and emerging therapies.

“Novel antibodies define clinical phenotypic subsets of disease,” he stated. Relevant antibodies include anti-MDA5, anti-TIF1-gamma, and anti-NXP.

Evaluation for muscle involvement should include assessment for dysphagia and bulbar weakness, a muscle strength examination, and laboratory testing that may include creatine kinase, aldolase, lactate dehydrogenase, and AST/ALT. Additional testing may include electromyography, MRI, or muscle biopsy. Clinically amyopathic DM can require additional consideration when determining the extent of evaluation.

Pulmonary assessment may include a 6-minute walk test and pulmonary function testing with complete spirometry and diffusing capacity for carbon monoxide every 6 months to annually. Imaging can be considered according to individual risk.

Malignancy evaluation should incorporate a thorough medical history, physical examination, laboratory testing, and age-appropriate cancer screening. Additional testing may include CT imaging of the chest, abdomen, and pelvis and transvaginal pelvic ultrasound. Additional screening may continue for 3 to 5 years, particularly for ovarian cancer. These recommendations do not apply to juvenile DM.

Treatment should be individualized according to the severity of muscle disease and the presence of malignancy or ILD. Options include sun protection, topical therapies, systemic corticosteroids, methotrexate, mycophenolate mofetil, and intravenous immunoglobulin.

Emerging strategies include JAK inhibition for refractory skin disease, FcRn inhibition, and anti-interferon-beta therapy. TYK2 inhibition was characterized as “mechanistically promising.”

Reference
Merola J. Recognizing and managing dermatomyositis: from rash to systemic disease. Presented at: Masterclasses in Dermatology NP/PA Institute; October 17–18, 2026; Dallas, TX.