Janus kinase inhibitors in localized scleroderma: a systematic literature review

Background/aim: 

Reports on the use of Janus kinase (JAK) inhibitors in the treatment of localized scleroderma are increasing in the literature. In this review, we examined the published studies regarding the use of JAK inhibitors in patients with localized scleroderma.

Materials and methods: 

We searched MEDLINE and Scopus for articles on patients with localized scleroderma treated with JAK inhibitors. The search included articles from the inception of these databases through August 1^st, 2024. 

Results: 

Our literature search showed 11 articles describing 17 patients with localized scleroderma treated with JAK inhibitors. Generalized morphea (47.1%) was the most common type of localized scleroderma in patients treated with JAK inhibitors. The most frequently used JAK inhibitor was tofacitinib (64.7%). In some cases, baricitinib (17.6%) and ruxolitinib (17.6%) were also preferred. All JAK inhibitors were mainly preferred for the treatment of resistant/progressive skin disease in these patients (60.7%). The improvement rate associated with JAK inhibitors was 88.2%. Relapse occurred in 33.3% of patients treated with JAK inhibitors. Side effects were reported in 18.2% of patients: one patient was diagnosed to have diffuse large B-cell lymphoma (n = 1) while on tofacitinib. 

Conclusion: 

JAK inhibitors could be considered as a therapeutic option, especially in patients with refractory localized scleroderma, but more extensive clinical trials are needed to clarify questions regarding their efficacy and safety data.