• Rebecca De Lorenzo, Clara Sciorati, Antonella Monno, Silvia Cavalli, Francesco Bonomi, Stefano Tronci, Stefano Previtali, and Patrizia Rovere-Querini.
• Università Vita-Salute San Raffaele.
• Medicine (Baltimore). 2021 Mar 5; 100 (9): e24372e24372.

RationaleSevere refractory idiopathic inflammatory myopathy (IIM) represents a challenge for the clinician. The lack of efficacy of available tools reflects our incomplete insight into the molecular events sustaining the inflammatory tissue damage in these patients. We present the first case of refractory IIM treated with anti-dipeptidyl peptidase-4 (DPP-4)/cluster of differentiation 26 (CD26) monoclonal antibody.Patient ConcernsA 55-year old man presented with proximal muscle weakness, diffuse erythematous skin lesions which rapidly evolved into ulcerations, dysphagia and dysphonia.DiagnosisIncreased serum creatine kinase levels and histological findings at muscle and skin biopsies were compatible with the diagnosis of dermatomyositis (DM). Several lines of treatment failed to control the disease including steroids, mycophenolate mofetil, tacrolimus, intravenous immunoglobulins and rituximab. Despite therapy, the patient also had recurrent intestinal vasculitis causing bowel perforation. Concurrently, DPP-4/CD26 expression in the patient's skin and skeletal muscle was observed.InterventionsThe patient was treated with begelomab, a murine immunoglobulin G2b monoclonal antibody against DPP-4/CD26.OutcomesDysphagia, skin lesions and intestinal vasculitis resolved and the patient experienced a significant improvement of his quality of life.ConclusionBlockade of DPP-4/CD26, which is expressed on T cells and mediates T cell activation and function, is safe and might be effective in patients with refractory DM.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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