Is there evidence for vasculitis in systemic sclerosis?
Systemic sclerosis (SSc) is a devastating and potentially life-threatening multi-organ system disease. SSc is marked by skin thickening and tightening, Raynaud's phenomenon and digital ischemia with ulceration, gastrointestinal dysmotility, cardiopulmonary involvement with pulmonary fibrosis and pulmonary arterial hypertension, as well as renal failure. Fibrosis is the most obvious manifestation of SSc. Vascular involvement and inflammation are other prominent components of SSc pathology, and both features are also seen in vasculitis. This review analyzes whether there is evidence for vasculitis especially with particular organ manifestations and subgroups of patients.
LSU Scleroderma and Sarcoidosis Patient Care and Research Center, Louisiana State University Health Sciences Center, New Orleans, LA, USA. ctd.ild@gmail.com
Lesley Ann
LA
Saketkoo
Oliver
O
Distler
D006801:Humans; D007249:Inflammation / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000503:physiopathology; D014652:Vascular Diseases / Q000209:etiology / Q000503:physiopathology; D014657:Vasculitis / Q000209:etiology* / Q000503:physiopathology