Risk of a systemic connective tissue disease in patients with Raynaud phenomenon

Margaret J. Larché is a professor of medicine in the Division of Rheumatology at McMaster University and director of the Canadian Scleroderma Research Group (CSRG).

What is the risk of a systemic connective tissue disease in persons with Raynaud phenomenon? Does the prognostic value of Raynaud phenomenon differ between women and men?

The risk of developing a connective tissue disease or a secondary Raynaud in somebody with Raynaud disease really depends on a few things. It depends on these red flags that I was mentioning earlier.

The age of presentation: most people who get primary Raynaud are young women, up to 10% of young women. It occurs at the time of adolescence usually. If you present as a 40-year-old woman with new Raynaud and never had it before, then I would be more suspicious of there being an underlying cause for this disease. I would be taking a much more thorough history and really being vigilant for a secondary cause.

Then, if on examination there are features such as puffy hands, digital ulcers, dilated nailfold capillaries, that would be really suspicious. What we know from several studies is that the more of those features you have, you’re much more likely to develop a connective tissue disease, such as systemic sclerosis. For example, if you have puffy fingers and dilated nailfold capillaries, and a positive antinuclear antibody (ANA) [titer], you’re 80% to 90% likely to get systemic sclerosis in the next 5 years. Those are the patients I really look out for.

Is there a predominance of women versus men? Yes, I worry more about men who get Raynaud, especially older men. I always hunt hard for either a rheumatologic or connective tissue disease cause or a vascular cause in those patients.