Janus kinase inhibitors therapy in idiopathic inflammatory myopathies: a case series
Abstract
OBJECTIVES
To evaluate the clinical response of Janus kinase inhibitors (JAKi) across subtypes of idiopathic inflammatory myopathies (IIM) in a cohort of patients with refractory disease.
METHODS
We conducted a retrospective analysis of all adult IIM patients treated with JAKi at our centre. Treatment response was assessed based on changes in muscle strength, serum biomarkers of muscle damage and inflammation, pulmonary function, radiological evolution of interstitial lung disease (ILD) and corticosteroid dosage.
RESULTS
Ten IIM patients who were previously or currently receiving JAKi therapy were identified. Six patients were female and the mean age was 52.7 years (standard deviation [SD] 13.91). Anti-synthetase syndrome was the most common subtype (n=5, 50%). At baseline, median manual muscle test 8 score was 136 (interquartile range [IQR] 25.5) and, after therapy, was 147 (IQR 8), representing a statistically significant increase (p<0.05). Corticosteroid dose reduction was also statistically significant (p<0.05), with the median daily dose decreasing from 10mg to 2.5mg. Additionally, four patients were able to discontinue corticosteroid therapy. In six patients with ILD, diffusion capacity for carbon monoxide improved significantly (p<0.01), from 68.33% (IQR 19.31) to 93% (IQR 7.27). No significant changes were observed in serum inflammatory markers, creatine kinase, forced expiratory volume first second or forced vital capacity.
CONCLUSIONS
JAKi therapy appears to be…
How strong is the evidence?
Evidence Hub reads this as a case series, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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