Systemic Sclerosis (Scleroderma) and Raynaud’s Phenomenon

• Systemic sclerosis (SSc; scleroderma) is a multiorgan autoimmune rheumatic disease characterized by fibrosis of the skin and internal organs, vascular abnormalities, and the production of autoantibodies. • SSc is divided generally into limited and diffuse forms based on the extent of skin involvement. • Diffuse SSc is characterized by more extensive skin involvement compared to limited SSc and is more likely to be associated with renal crisis, pulmonary fibrosis, cardiac involvement, and decreased survival. • Prognosis is worse in SSc than in other rheumatic diseases and the risk of mortality is high (3–5 times greater than that of an age and sex-matched population (Pauling et al. 2021; Ferri et al. 2002)). • Earlier disease detection and improvements in the treatment of SSc-related organ involvement (especially renal disease) have contributed to improved survival. • Pulmonary disease remains a major source of morbidity and mortality for patients with SSc. Pulmonary fibrosis and pulmonary hypertension can occur in both major subsets, but interstitial fibrosis is more common in diffuse SSc. Patients with limited SSc are predisposed to the development of pulmonary arterial hypertension (PAH). • Autoantibody profiles used in diagnosis can also predict risk and pattern of organ involvement that can be expected in SSc. • Raynaud’s phenomenon (RP) is characterized by vasospasm of the digital arteries that leads to well-demarcated pallor with cyanosis and rubor. • RP, which is essentially universal among patients with SSc, is the first symptom in the majority of patients. • Primary RP is often mild and may not require pharmacological treatment. In secondary RP associated with SSc, however, the vasospasm typical of primary RP is also accompanied by structural blood vessel problems that lead to luminal narrowing. Digital ischemia with RP associated with SSc is often more severe than in primary RP. • Complications of RP in SSc can include digital ulcers associated with pain and functional disability. These are often complicated by infection and sometimes lead to amputation. • Diffuse SSc often presents with puffiness of the hands associated with pain in a way that mimics rheumatoid arthritis at early stages.