The Lack of a Durable Response to Tofacitinib in Anti-PL-12 Anti-synthetase Syndrome With Progressive Interstitial Pneumonitis

Abstract

We discuss a case of a 48-year-old female with anti-PL-12 anti-synthetase syndrome. She presented with dermatitis and myositis and developed rapidly progressive interstitial lung disease (ILD) while on prednisone and azathioprine, which responded dramatically to the addition of tofacitinib. However, the patient later developed arthritis, worsening skin disease, cutaneous vasculitis, and worsening ILD with corticosteroid reduction. The response to a standard dose of tofacitinib, although dramatic in the subacute phase of ILD, was not durable in terms of preventing a flare-up of skin disease, cutaneous vasculitis, arthritis, and the slow progression of pulmonary fibrosis as corticosteroids were tapered. Rituximab and nintedanib were then sequentially added to her therapy. Our experience suggests that rather than sequential addition, targeted triple therapy with tofacitinib, rituximab, and an antifibrotic should be considered early in the disease course in patients with dermatomyositis and severe ILD.