Vasospastic condition characterised by episodic attacks of colour changes that are well demarcated and associated with numbness and pain in the digits on exposure to cold.
Prevalence of 3-4% of population (up to 30% in some cold climates).
Can be primary or secondary to another condition.
Primary Raynaud’s Disease (described by Raynaud in 1888):
No underlying disease process (in some case Raynaud’s may be presenting feature by up to a number of years for several diseases). Affects 4-30% of the population. Higher prevalence in cooler climates.
F4-9x>M. Onset usually ages 15-45 years. Increased frequency of migraine headache.
Secondary Raynaud’s Disease:
Due to – rheumatological diseases (SLE (in up to 50%), scleroderma/systemic sclerosis (in 90%), polymyositis/dermatomyositis, Sjogren’s syndrome, rheumatoid arthritis, vasculitis, fibromyalgia, MCTD); drugs (beta-adrenoceptor antagonist (most common), ergot (stimulates alpha-adrenoceptors), bleomycin, methysergide); occupational/traumatic related (use of tools that vibrate (can occur in 30-80%) – eg rock drillers, jack/pneumatic hammers, chain saws); vascular diseases (postembolism, thoracic outlet syndrome); hyperviscosity diseases (polycythemia, cryoglobulinaemia, leukaemia, thrombocytosis); complex regional pain syndrome; carpal tunnel syndrome; hypothyroidism (up to 20%)
Acute vasospasm reduction in digital blood flow.
Cause of vasospasm unclear – may be:
a) Hyperactivity of sympathetic nervous system
b) Dysfunction of local regulation of vascular tone (eg increased responsiveness of peripheral alpha-adrenergic receptors; deficiency of vasodilatory agents or resistance to agents; increased levels of vasoconstrictors)
In secondary Raynaud’s – an intrinsic vascular abnormality increases the risk of closure of digital vessel.
Triphasic colour response/stages (all three stages not always seen in all patients):
1) Pallor/blanching (sometimes yellowish and numb) – usually well demarcated
2) Cyanosis from deoxygenation/desaturation of static venous blood
3) Rubor from reactive hyperaemia as blood vessel open may get throbbing pain
Hands more commonly affected (usually spares thumb) – 40-50% of cases toes are also affected. Less frequently affects the nose, tongue and ears. Some have excessive sweating of the hands. Peripheral pulses are almost always present.
Cold exposure is most common precipitant – however can be precipitated by emotion, trauma, hormones, smoking
Most only need preventative measures – avoidance of cold exposure
Some benefit from biofeedback and self-hypnosis
Pharmacological if severe – nifedipine, reserpine, guanethidine, prazosin
Surgical sympathectomy can be considered if severe and unresponsive to conservative measures.