Raynaud’s phenomenon is a vasospastic disorder that affects over 9 million people in the USA (National Institute of Arthritis and Musculoskeletal and Skin Diseases, 2006). Maurice Reynaud’s description of arterial insufficiency to the fingers in 1862 led to his name being the eponym of this condition. He described the process as a “local asphyxia of the extremities” as a result of “increased irritability of the central parts of the cord presiding over vascular innervation.” Raynaud’s phenomenon is nine times more common in females and typically occurs between the ages of 15–40 years. These patients have an exaggerated vasoconstriction of their digital arteries in response to certain environmental triggers, which leads to pale, cold, numb and sometimes painful digits. These symptoms can last minutes to hours and may reoccur several times through the day. For the majority of patients, the symptoms are simply bothersome, but for 20% the symptoms are so severe that they seek medical attention. The digital artery vasospasm leads to diminished blood supply to the fingertips, which causes pain, ulcerations and disuse. Many patients require digit amputations for recalcitrant ulcers or exposure of the distal phalanx. The resultant digit ischemia may be also associated with considerable morbidity associated with loss of function, disability and depression.
Primary Raynaud’s disease is an idiopathic condition, and secondary Raynaud’s disease is associated with other connective tissue disorders. Secondary Raynaud’s disease affects 90% of patients with scleroderma, 90% of patients with mixed connective tissue disease, 33% of patients with lupus and 33% of people with Sjögren’s syndrome. The pathophysiology of primary versus secondary Raynaud’s disease is likely different, with secondary Raynaud’s disease invariably causing more severe and debilitating symptoms (Figs. 23.1 and 23.2).