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Systemic sclerosis, also termed scleroderma, is a multisystem connective tissue disease involving autoimmunity, inflammation, fibrosis and vasculopathy. The clinical presentation of systemic sclerosis is characterized by skin thickening, Raynaud's phenomenon, vasculopathy-induced tissue death and fibrosis of internal organs.
Here, the authors identify six risk SNPs for systemic sclerosis by performing GWAS on Japanese patients and meta-analyzing Japanese and European GWAS datasets. Downstream analyses indicate that B cells contribute to pathogenesis.
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.
New research suggests that A20 expressed in fibroblasts protects against fibrosis (whereas its negative regulator downstream regulatory element antagonist modulator (DREAM) promotes fibrosis), and highlights the therapeutic potential of targeting the A20–DREAM network.
A new study has identified an association of Thy-1 expression with skin fibrosis in systemic sclerosis, and has shown that Thy1 knockout in mice attenuates bleomycin-induced skin fibrosis.
Do you inform patients about risks and chances of success associated with different choices when treatment needs to be changed? This approach takes time and requires patient health literacy. Results from a discrete-choice experiment in scleroderma lung disease add to the debate on shared decision-making.
