Connective tissue diseases articles from across Nature Portfolio

Interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) is associated with considerable morbidity and mortality. In this Review, various unmet needs in the management of SSc–ILD are discussed, and solutions are proposed to help improve outcomes for these patients.

The autoimmune disease systemic sclerosis is characterized by fibrosis, which is poorly targeted by the use of anti-inflammatory and immunosuppressive drugs. In this Review, the authors describe the fibrotic mechanisms underlying SSc and attempts to develop drugs to specifically target the fibrotic extracellular matrix.

In the LUMINA-1 trial for fibrodysplasia ossificans progressiva, garetosmab, an activin A monoclonal antibody, did not lead to significant changes in heterotopic ossification lesion activity in pre-existing lesions in period 1. Garetosmab prevented the formation of new lesions in both periods 1 and 2.

Chromatin accessibility of an enhancer that regulates the expression of a disease-associated microRNA is affected in monocytes from patients with systemic lupus erythematosus, highlighting this enhancer as a potential therapeutic target.

New research provides insights into the mechanisms underlying the renal protective effects of sodium glucose cotransporter 2 inhibitors in lupus nephritis.

No drugs are yet approved for the treatment of primary Sjögren syndrome, despite a large number of clinical trials. Non-conventional approaches can help to identify novel therapeutic targets. Using a drug-repositioning transcriptomic approach, interferon has emerged once again as a major target for intervention in this disease process.

Clinical and molecular analysis of autoantibodies against the endonuclease DNase1L3 in patients with systemic lupus erythematosus led to the identification of a subset of pathogenetic antibodies that are cross-reactive with double-stranded DNA.