Published online Nov 12, 2015. doi: 10.5499/wjr.v5.i3.131
Peer-review started: March 31, 2015
First decision: April 27, 2015
Revised: June 26, 2015
Accepted: July 11, 2015
Article in press: July 14, 2015
Published online: November 12, 2015
Scleroderma (or systemic sclerosis) is a rare disease associated with significant morbidity and mortality. Although previously thought to have a uniformly poor prognosis, the outlook has changed in recent years. We review recent insights into the pathogenesis, clinical features, assessment and management of scleroderma.
Core tip: Scleroderma is a rare disease associated with significant morbidity and mortality. Better understanding regarding its pathogenesis has led to exploration of various newer therapeutic targets. Anti B cell therapy, endothelin receptor antagonists, phosphodiesterase-5 (PDE-5) inhibitors and autologous stem cell transplant holds promise in the management of systemic sclerosis. PDE-5 inhibitors in particular have potential to be disease modifying agents as they not only improve Raynaud’s phenomenon, but also heal digital ulcers, improve pulmonary hypertension and in addition by virtue of antifibrotic properties may have beneficial effect on skin and lung fibrosis.