SNOWMASS, COLO.– Physical examination of the hands yields strong clues that a patient presenting with sharply demarcated color changes of the digits has primary Raynaud’s phenomenon due to reversible small-vessel vasospasm rather than secondary Raynaud’s involving vasospasm plus structural disease in the microcirculation, Dr. Fredrick M. Wigley said at the Winter Rheumatology Symposium sponsored by the American College of Rheumatology.
Primary Raynaud’s phenomenon is precipitated by cold temperatures or emotional stress. The attacks are symmetric and involve all the fingers on both hands. The pallor doesn’t extend beyond the metacarpophalangeal joints into the palms; if it does, this is not just a digital arteriolar vasospastic event, but rather one involving compromise of larger vessels, as occurs in Raynaud’s from a secondary cause such as scleroderma, said Dr. Wigley, professor of medicine, associate head of rheumatology, and director of the scleroderma center at Johns Hopkins University, Baltimore.
Examination of the nailfold capillaries under magnification using immersion oil is informative, he added. Normal nailfold capillaries are consistent with primary Raynaud’s phenomenon. But abnormal nailfolds in a patient with no history or physical findings suggestive of a secondary cause of Raynaud’s are associated with a 20% chance of developing a rheumatic disease – most often scleroderma – within the next 2 years. And if the nailfold capillaries are abnormal and the patient also has anticentromere or other autoantibodies, the 2-year likelihood of a rheumatic disease diagnosis jumps to 80% (Rheum. Dis. Clin. North Am. 2008;34:89-114).
Patients with primary Raynaud’s phenomenon often show a livedo reticularis pattern of mottled, purplish, lacelike vessels on their skin, especially on the legs. The thermoregulatory shunts present in the blood vessels of patients with primary Raynaud’s phenomenon are abnormally sensitive to sympathetic tone, and the livedo reticularis is believed to be another consequence of these upregulated circulatory shunts.
A major clinical distinction between primary and secondary Raynaud’s is that patients with primary Raynaud’s phenomenon do not get digital ulcers, gangrene, or signs of tissue injury.
Only about one-third of scleroderma patients with severe secondary Raynaud’s develop ischemic digital ulcers. That’s surprising because scleroderma patients with Raynaud’s have intimal proliferation and luminal narrowing of their digital vessels, making them prone to occlusion, along with reduced levels of endogenous vasodilators and elevated levels of endothelin-1 and other vasoconstrictors that render their vascular endothelial layer easily provoked into spasm.
“If you do an angiogram – and I generally don’t – you’ll be frightened because there’s an incredible amount of disease present despite the fact that many patients don’t get ischemic ulcers,” Dr. Wigley said.
In the two-thirds of patients with scleroderma and severe Raynaud’s phenomenon attacks who don’t get ulcers, it’s “certainly reasonable” to manage their digital disease as if they had primary Raynaud’s, according to the rheumatologist.
One quick way to predict whether a scleroderma patient with severe, dramatically cyanotic Raynaud’s phenomenon is likely to develop worrisome digital ulcers is simply to press on the cold fingers, then release them.
“If you see a good, rapid flush you’ve got good blood flow and you’re probably not going to get into trouble with an ischemic event,” Dr. Wigley said.
Dr. Wigley reported serving as a consultant to Novartis and United Therapeutics and receiving research grants from KineMed, MedImmune, and CSL Behring.