Long-lasting improvement of refractory antisynthetase syndrome with tocilizumab: a report of two cases

Abstract

No standardised and universal treatment is available for antisynthetase syndrome. In particular, there is an unmet need for a single efficient treatment acting on its various manifestations, including interstitial lung disease, myositis and polyarthritis.We describe the cases of two patients with multiple and severe manifestations, including joint, muscular and lung involvement, both refractory to various treatments, including rituximab, who demonstrated significant improvement of all their manifestations, including joint, muscular and lung diseases on tocilizumab. The response was also long-lasting, with both patients still being in full remission after >10 years of treatment.Our experience shows that interleukin-6 inhibition could be a very effective treatment option in antisynthetase syndrome, with efficacy on a wide spectrum of manifestations.

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease

“…A systematic literature review and case series demonstrated a beneficial role of IL-1 inhibition with anakinra in seven patients with difficult to treat manifestations of ASyS, despite the use of other immunosuppressive agents [134]. Similarly, IL-6 blockade with tocilizumab in two patients with treatment-refractory ASyS-ILD showed an improvement in and stabilization of lung function over time [135]. Abatacept (CTLA4-Ig) used in eight patients with ASyS-ILD refractory to other therapeutic agents was well tolerated and showed improvement in FVC and DLCO [136].…”