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Improvement of cutaneous inflammation and panniculitis in patients with dermatomyositis by the Janus kinase inhibitor baricitinib

Dear Editor, Dermatomyositis (DM) is a rare autoimmune disease with cutaneous and systemic involvement significantly impairing quality of life. Current treatment options are unspecific and often induce only partial remission, especially in DM‐associated panniculitis. The pathogenesis of the disease is fairly unknown; however, induction of type I interferon (IFN) and its IFN‐stimulated genes (ISGs) was discovered in patients’ blood, muscle and skin.1 Inhibiting the activation of Janus kinase (JAK)‐transmitting signals of the type I IFN receptor is a valuable therapeutic option, and the efficacy of the JAK1 and JAK2 inhibitor baricitinib was reported in eight patients with juvenile DM and in two patients with adult DM.2–6

The prominent type I IFN signature in DM prompted us to treat three patients with classic adult DM with the JAK inhibitor baricitinib.1 Patient 1 had experienced a recurrent disease course for 25 years. She had previously received immunosuppressive therapy and intravenous immunoglobulin (IVIG). Partial remission was achieved with low‐dose prednisolone (5 mg per day), methotrexate, hydroxychloroquine and adalimumab. Nevertheless, she presented with a new disease flare with violaceous erythema prone to the face, décolleté, neck and periungual area, but normal muscle enzymes (Figure 1a). The therapy with methotrexate and adalimumab was terminated due to an increase in liver enzymes. Thereafter, tofacitinib was started but was not well tolerated because of lactose intolerance in the patient. Therefore, the therapy was changed to baricitinib 4 mg daily. The neck, facial and periungual erythema completely regressed within 5 months of treatment (Figure 1a).

Identiferoai:union.ndltd.org:DRESDEN/oai:qucosa:de:qucosa:92097
Date14 June 2024
CreatorsFischer, Kristina, Aringer, Martin, Steininger, Julian, Heil, Julius, Beissert, Stefan, Abraham, Susanne, Günther, Claudia
PublisherOxford University Press
Source SetsHochschulschriftenserver (HSSS) der SLUB Dresden
LanguageEnglish
Detected LanguageEnglish
Typeinfo:eu-repo/semantics/publishedVersion, doc-type:article, info:eu-repo/semantics/article, doc-type:Text
Rightsinfo:eu-repo/semantics/openAccess
Relation1365-2133, 10.1111/bjd.21252

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