Raynaud’s disease is a reversible ischemic event that takes place in the extremities of the body in response to various stimuli such as exposure to cold and stress. It is named after the French physician Maurice Raynaud. Raynaud’s disease is most often a benign condition and may disappear in a substantial proportion of subjects. The prevalence of the disease is approxamately 5 to 6 percent; however, the prevalence varies in populations. The disease is more common in females than males.
The exact etiology of Raynaud’s disease is undetermined; however, many of the risk factors have been established. In addition to being a triggering factor for the disease, cold temperatures also appear to be an etiological factor in the pathogenesis of the disease. The exposure to cold temperatures trigger the loss of heat from the body; the small arteries in the extremities are narrowed and thus have a restricted blood supply; this leads to preservation of the body’s temperature for other vital organs.
In Raynaud’s disease, this normal process is exaggerated, further leading to excessive constriction of arteries and thus vasospasm, leading to interruption of blood flow to the extremities and hence a pallor of the skin, cyanosis (due to desaturation of hemoglobin), and numbness. As the arteries return to their normal dilated state, an increased blood flow occurs (hyperema), and hence, redness of skin (erythema), followed by pain in the extremeties. Although most typically noted in the fingers, the circulation of the toes, ears, nose, and tongue are also frequently affected.
Emotional stress alone can cause an episode of Raynaud’s. If the cause of the disease is attributed to another disorder (most commonly an autoimmune disorder), the name of the condition is referred as Raynaud’s phenomenon or secondary Raynaud. Autoimmune diseases include rheumatoid arthritis, systemic lupus erythematosis, Sjögren’s syndrome, and scleroderma. Other diseases include hepatitis B and C; neoplasm’s such as lymphoma and myeloma; and metabolic syndromes such as acromegaly.
Other potential causal or triggering factors can be found in the workplace such as the use of vibrating tools; this is commonly seen in the food processing industry. Neurovascular syndromes such as carpal tunnel syndrome may exacerbate symptoms and should be considered particularly in patients with asymmetrical digital lesions.
In some, the disease may be the first sign of a connective tissue disease (CTD) such as scleroderma. Risk factors for developing CTDs include the severity of the disease at onset, a positive test for antinuclear antibodies, and age at onset. An abnormal nailfold capillary pattern is strongly associated with the subsequent development of a CTD. Some studies have suggested that risk factors for Raynaud’s phenomenon differ between men and women, for example, age and smoking are associated with the disease in men only, while the associations of marital status and alcohol use with the disease are observed in women.
Diagnosis of the disease and classification into primary and secondary is made using a criteria system. The criteria for primary disease include symmetric attacks, the absence of tissue necrosis, ulceration or gangrene, the absence of a secondary cause, negative antinuclear antibodies, normal nailfold capillaroscopy, and a normal erythrocyte sedimentation rate. Secondary disease is characterized by an age of onset of more than 30 years, painful and asymmetric attacks, ischemic skin lesions, positive antinuclear antibodies, capillaroscopic abnormalities, and clinical features which suggest CTDs.
Diagnosis is made using history and examination, chest radiograph and barium esophagram, pulmonary function tests, antinuclear and anticentromere antibodies test, cryoglobulins test, and nailfold capillary microscopy (technique used in childhood and adults for morphofunctional study of peripheral microcirculation). Treatment for Raynaud’s disease includes peripheral sympathectomy which improves perfusion to ischemic digits and enables amputation to be avoided. The ingestion of omega-3 fatty acids could benefit patients with Raynaud’s phenomenon as fatty acids induce a favorable vascular response to ischemia. Ingestion of fish oil improves tolerance to cold exposure and delays the onset of vasospasm in patients with primary Raynaud’s disease.
Management of the disease includes physical therapy, avoidance of triggers, and a variety of medications, depending on the disease severity. For primary and uncomplicated secondary disease, calcium channel blockers (e.g., nifedipine), particularly ones that cause vasodilatation, are used; angiotensin converting enzyme inhibitors such as losartan and prostaglandins analogues may also have benefit. Other agents including alpha receptor blockers, for example, prazosin; sympatholytic agents, for example, reserpine; and selective serotonin-reuptake inhibitors, for example, fluoxetine may be useful. Secondary Raynaud’s is treated according to the underlying disorder. Prognosis for primary Raynaud’s syndrome is good; however, patients with secondary symptoms require greater care and treatment strategies to avoid further complications.
Connective Tissue Disorders.