Ansprechen einer Dermatomyositis mit Lungenbeteiligung auf eine Januskinase-Inhibitor-Therapie

Hörnig, Juliane; Weinhage, Toni; Schmidt, LH; Buerke, Boris; Schneider, Udo; Pavenstädt, Hermann; Becker, H; Gabriëls, Gert

doi:10.1007/s00393-018-0565-8

Cited by 28 publications

(14 citation statements)

References 10 publications

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“…More recently, different case reports and small case studies showed a positive outcome of recalcitrant chronic DM after treatment with either ruxolitinib or tofacitinib (79–82) (Figure 3). Remarkably, JAKi seem to have a positive effect on lung involvement in DM (79, 82–84), which often represents a high-risk-mortality complication. The efficacy of tofacitinib is currently under investigation in a small cohort of 10 patients with recalcitrant DM (NCT03002649) (Table 4).…”

Section: Dermatomyositismentioning

confidence: 99%

Emerging Topical and Systemic JAK Inhibitors in Dermatology

2019

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Accumulating data on cellular and molecular pathways help to develop novel therapeutic strategies in skin inflammation and autoimmunity. Examples are psoriasis and atopic dermatitis, two clinically and immunologically well-defined disorders. Here, the elucidation of key pathogenic factors such as IL-17A/IL-23 on the one hand and IL-4/IL-13 on the other hand profoundly changed our therapeutic practice. The knowledge on intracellular pathways and governing factors is shifting our attention to new druggable molecules. Multiple cytokine receptors signal through Janus kinases (JAKs) and associated signal transducer and activators of transcription (STATs). Inhibition of JAKs can simultaneously block the function of multiple cytokines. Therefore, JAK inhibitors (JAKi) are emerging as a new class of drugs, which in dermatology can either be used systemically as oral drugs or locally in topical formulations. Inhibition of JAKs has been shown to be effective in various skin disorders. The first oral JAKi have been recently approved for the treatment of rheumatoid arthritis and psoriatic arthritis. Currently, multiple inhibitors of the JAK/STAT pathway are being investigated for skin diseases like alopecia areata, atopic dermatitis, dermatomyositis, graft-versus-host-disease, hidradenitis suppurativa, lichen planus, lupus erythematosus, psoriasis, and vitiligo. Here, we aim to discuss the immunological basis and current stage of development of JAKi in dermatology.

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Section: Dermatomyositismentioning

confidence: 99%

Emerging Topical and Systemic JAK Inhibitors in Dermatology

2019

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“…In comparison, six patients receiving only triple therapy (historical controls) died within 2 months (14). Baseline FVC% or DLCO% measurements were reported for 23 patients with DM-ILD, and improvements, although not explicitly quantified in all cases, were noted in all 23 patients treated with JAK inhibitors (23,33,38,(41)(42)(43).…”

Section: Interstitial Lung Diseasementioning

confidence: 99%

Use of Janus kinase inhibitors in dermatomyositis: a systematic literature review

Paik

Lubin

Gromatzky

et al. 2022

Clinical and Experimental Rheumatology

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Dermatomyositis (DM) is an idiopathic inflammatory myopathy that commonly manifests with proximal muscle weakness and is associated with extramuscular pathology, including characteristic skin lesions such as Gottron's papules and heliotrope rash, as well as lung, gastrointestinal, joint, and cardiac involvement. Systemic corticosteroids are a cornerstone of therapy, and more recently intravenous immunoglobulin (IVIG; OCTAGAM ® ) has been approved by the US Food and Drug Administration for the treatment of adults with DM. Both steroids and IVIG represent nonspecific anti-inflammatory therapy, and more targeted approaches are lacking. Transcriptomics has identified upregulation of interferon (IFN)-regulated genes as key features of both adult DM and juvenile DM (JDM). Accordingly, blocking IFN signalling through inhibition of the Janus kinase (JAK) pathway represents a potential treatment option for DM. Placebo-controlled trial data assessing the use of JAK inhibitors for the treatment of DM are limited; as such, a systematic literature review was undertaken to assess the evidence of JAK inhibitors in the treatment of patients with DM. Terms related to DM and JAK inhibitors were searched using PubMed, Embase, Web of Science, Scopus, and Dimensions to identify peer-reviewed publications reporting patients with DM who were treated with a JAK inhibitor. Baseline demographics, clinical characteristics, and treatment outcome data were extracted. A total of 48 publications reporting 145 unique patients (adult DM, n=84; JDM, n=61) were identified. Among cases of adult DM, 61 of 84 (73%) had refractory skin disease at baseline, and all (61 of 61) reported improvement in cutaneous symptoms. Of patients with a wide range of DM manifestations, including skin lesions, muscle weakness, and ILD. Our systematic literature review suggests that JAK inhibitors may be a viable treatment option for DM/ JDM, and randomised controlled trials are necessary to confirm these findings.

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“…Hierzu liegt eine große Fallserie vor, die einen positiven Einfluss auf RA und ILD gezeigt hat [57]. Bei SSc-ILD konnte in 2 Studien eine mögliche Wirksamkeit von Tocilizumab bei SSc-ILD gezeigt werden [58,59]. [53] das einen Einsatz von Pirfenidon bei SSc-ILD vertretbar erscheinen lässt [62].…”

Section: Biologikaunclassified

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