Upadacitinib for refractory cutaneous disease in a patient with tif1γ-positive dermatomyositis
Year: 2026
Type: article
Abstract: Upadacitinib for refractory cutaneous disease in a patient with tif1␥-positive dermatomyositis ଝ Dear Editor, A 49-year-old woman presented facial and truncal erythema, progressive proximal weakness, and dysphagia.Physical examination showed typical signs of classic dermatomyositis, including heliotrope rash, Gottron papules over the metacarpophalangeal and interphalangeal joints, and V-sign erythema (Fig. 1).Serum creatine kinase level was elevated at 730 U/L (<90 U/L).The myositisspecific antibody panel was positive for anti-Transcription Intermediary Factor 1-gamma (TIF1␥) antibody.Given the malignancy risk associated with TIF1␥ positivity, cancer screening was performed according to the International Guideline for Idiopathic Inflammatory Myopathy-Associated Cancer Screening.2 This included a gynecological examination, serum tumor markers, mammography, colonoscopy, cross-sectional Computed Tomography (CT) imaging of the neck, thorax, abdomen, and pelvis, and Positron Emission Tomography-CT, which were all negative.Baseline assessment showed a Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) activity score of 26, consistent with moderate-to-severe cutaneous activity, and muscle weakness with Manual Muscle Testing-8 (MMT-8) score of 64/80.Treatment with prednisolone (1 mg/kg/day) and mycophenolate mofetil (2 g/day) was started.After 4months, rituximab was added due to inadequate response in both cutaneous and muscular domains.However, two courses of rituximab failed to achieve remission.Intravenous immunoglobulin was inaccessible due to reimbursement limitations, and corticosteroid dosing regimens were limited by the compression fracture.Three months after the last rituximab infusion, upadacitinib, a selective Janus Kinase 1 (JAK1) inhibitor, was
Institutions: Istanbul University-Cerrahpaşa, Istanbul University
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $1,700
OpenAlex ID: W7208711594