Targeted therapies in systemic sclerosis, myositis, antiphospholipid syndrome, and Sjögren's syndrome.

Targeted therapies using biological disease-modifying antirheumatic drugs (bDMARDs) and small molecule synthetic drugs have revolutionized rheumatological practice. Initially developed for the treatment of immune arthritis (rheumatoid arthritis, psoriatic arthritis, and spondylarthritis), both bDMARDs and small molecule synthetic drugs are now increasingly entering the space of connective tissue disease (CTD) treatment. Recent clinical trial data in systemic sclerosis (SSc) have been particularly encouraging with positive effects on outcomes having been observed with nintedanib preventing the decline of lung function in patients with SSc-related interstitial lung disease. Randomized trials targeting B-cells by rituximab in primary Sjogren's syndrome have led to mixed results. Novel strategies to target B-cells in primary Sjögren's syndrome including ianalumab and belimumab are underway and will hopefully result in clear treatment effects. Inflammatory idiopathic myositis (polymyositis (PM) and dermatomyositis (DM)) and antiphospholid syndrome are proving to be more difficult to tackle but are nonetheless the subject of ongoing studies. To what extent new compounds can replace more traditional immunosuppressive drugs remains to be determined, but if the experience in immune arthritis has taught us anything it is that combination therapy may be the way to go.