A systematic literature review of Janus kinase inhibitors for the treatment of systemic sclerosis

Abstract

Objectives: The use of Janus kinase inhibitors is increasing in systemic sclerosis, a complex autoimmune disease characterized by fibrosis, vasculopathy, and immune dysregulation. In this review, we aimed to examine the studies in the literature reporting on patients with systemic sclerosis treated with Janus kinase inhibitors.

Methods: We performed a search on MEDLINE and Scopus for articles involving patients with systemic sclerosis treated with Janus kinase inhibitors from the inception of these databases through 1 August 2024.

Results: Our literature search revealed 18 articles describing 87 systemic sclerosis patients treated with Janus kinase inhibitors. The median (min-max) age of these patients was 48.5 (13-78) years (F/M = 4.9). The most commonly used Janus kinase inhibitors in systemic sclerosis were tofacitinib (82.8%), followed by baricitinib (13.8%). Janus kinase inhibitors were most commonly used to treat the combination of interstitial lung disease and skin involvement (44.9%), and the combination of gastrointestinal system and skin involvement (38.8%). Improvement rates with Janus kinase inhibitors were 87.5%, and 5.9% of patients on Janus kinase inhibitors relapsed. Adverse events were observed in 50% of systemic sclerosis patients treated with Janus kinase inhibitors. Various infections (21.1%) were the most common adverse event reported following Janus kinase inhibitor use.

Conclusion: Although Janus kinase inhibitors seem to be very effective, especially in patients with systemic sclerosis who have resistant/progressive skin disease, some side effects should not be ignored. Therefore, controlled clinical trials in larger populations are needed on Janus kinase inhibitors use in systemic sclerosis.

Keywords: Janus kinase inhibitors; systemic sclerosis; tofacitinib; treatment.

Figure 1.

The PRISMA flow diagram of literature screening regarding Janus kinase inhibitor use in patients with systemic sclerosis. cGVHD, chronic graft-versus-host disease; JAK, Janus kinase. ^aOthers: guideline, letter, editorial, conference paper, short survey, comment, and note.

Figure 2.

The diagram presents the indications for Janus kinase inhibitors in the treatment of systemic sclerosis. GIS, gastrointestinal system; ILD, interstitial lung disease.