Raynaud's phenomenon and scleroderma
Raynaud phenomenon manifests as recurrent vasospasm of the fingers and toes and usually occurs in response to stress or cold exposure.
Secondary Raynaud phenomenon should be distinguished from primary Raynaud phenomenon (Raynaud disease). Raynaud disease is characterized by the occurrence of the vasospasm alone, with no association with another illness.
Diagnostic criteria for primary Raynaud phenomenon include the following:
- Attacks triggered by exposure to cold and/or stress
- Symmetric bilateral involvement
- Absence of necrosis
- Absence of a detectable underlying cause
- Normal capillaroscopy findings
- Normal laboratory findings for inflammation
- Absence of antinuclear factors
Primary Raynaud phenomenon is treated with lifestyle measures. Young women who have had Raynaud phenomenon alone for more than 2 years and have not developed any additional manifestations are at low risk for developing an autoimmune disease. The same should not be said for older patients and male patients with Raynaud phenomenon, as vasospastic symptoms may predate systemic disease by as many as 20 years.
Secondary Raynaud phenomenon is a designation usually used in the context of vasospasm associated with another illness, most commonly an autoimmune disease.The most common association is with systemic sclerosis (90% in individuals with scleroderma). Systemic sclerosis is a rare but severe disease associating autoimmunity, vasculopathy (digital ulcers, Raynaud phenomenon, pulmonary hypertension, renal crisis) and fibrosis (skin, lungs…). In the most severe forms of the disease morbimortality is very high and justify aggressive immunosuppressive treatments (cyclophosphamide, rituximab, heterologous stem cell transplant). Nevertheless none of these treatments has really reversed the process of the disease and a key point remains an early diagnosis to prevent damages. Raynaud phenomenon constitutes an important red flag in that approach.