Tacrolimus rescue therapy for severe respiratory failure in the anti-synthetase syndrome

Abstract

Interstitial lung disease (ILD) is the major determinant of morbidity and mortality in the anti-synthetase syndrome (ASS). The therapeutic efficacy of corticosteroids for the ILD component is limited; hence, additional immunosuppressive and immunomodulatory therapies have been tried with a modicum of success in recent years. Tacrolimus, a calcineurin inhibitor, is one potential therapy. We describe four consecutive patients with ASS whom we treated with tacrolimus at a quaternary referral hospital in 2009-2013. All four patients had significant ILD, three had severe and progressive ILD, and two had been referred for consideration of lung transplantation. Tacrolimus use was associated with improvement in ILD in all four patients with a mean follow-up of 3 years. Our case series adds further evidence to support the use of tacrolimus as salvage therapy for severe respiratory failure due to ILD in ASS, which may be associated with a dramatic and enduring response.