Practical Approach to the Patient with Raynaud’s Phenomenon: Primary Versus Secondary Raynaud’s Phenomenon

This chapter addresses the two main questions posed when a patient presents with Raynaud’s phenomenon (RP). First: ‘Why does this patient have RP? Is it primary (idiopathic) or secondary, and if secondary, to what?’ Second: ‘What treatment is required?’ Assessment of severity of RP is also discussed. Most patients with RP have primary RP (as illustrated in a case history), but a minority have RP secondary to an underlying disease/condition, for example, the connective tissue disease systemic sclerosis (SSc). Secondary RP can be very severe. A careful history and examination, together with some key investigations including testing for antinuclear antibody (ANA) and nailfold capillaroscopy, will usually reveal the diagnosis. Patients with primary RP should have no symptoms suggestive of an underlying disease and no abnormalities on examination: they should be ANA negative, and the full blood count, erythrocyte sedimentation rate, and nailfold capillaroscopy should all be normal. Most patients with primary RP can be reassured and do not require drug treatment which is, however, required in many patients with secondary RP. If drug treatment is required, a calcium channel blocker (sustained release) is generally the first choice, starting at low dosage and gradually increased as tolerated. A phosphodiesterase type 5 inhibitor is usually the second choice, especially in RP secondary to connective tissue disease. A number of other vasodilators can be tried, although the evidence base for other therapies is very weak, reflecting the challenges of clinical trials in RP. Predicting those patients who progress from ‘isolated’ to SSc-related RP is briefly discussed. ‘Red flags’ for an underlying SSc-spectrum disorder are puffy fingers, a positive ANA, a SSc-specific autoantibody, and abnormal nailfold capillaries.