Raynaud's disease, progressively Raynaud's Phenomenon (RP), is an exaggerated vascular response to the cold or to emotional stress, likely through abnormal vasoconstriction of digital arteries and arterioles.
It typically presents as well demarcated colour changes of the skin of the fingers and toes. The episodic nature of attacks means that patient self-reporting is often relied upon for diagnosis.
It may be considered primary as an isolated condition or secondary as a manifestation of a related illness such as Sjögren's syndrome, systemic sclerosis or systemic lupus erythematosus amongst others 1.
Mean prevalence 5%, usually presenting between the ages of 15 and 25. Known risk factors/associations include 2:
- Female gender
- Oestrogen replacement therapy
- A positive family history
- Manual labour
Classically, RP is diagnosed clinically based on digital colour changes – white (blanching due to vasoconstriction of arterioles), blue (cyanosis) and red (hyperaemia with reperfusion).
There may be associated pain, paraesthesia and dysfunction. Digital colour changes are usually symmetrical, with sparing of the thumbs. The ears, nose and nipples may also be affected. Diagnosis does not demand a triphasic discolouration and biphasic changes should still raise suspicion.
A variety of clinical and serological abnormalities can be found in patients with PRP and 1 in 8 will be ultimately be diagnosed with a connective tissue disease 3.
Secondary RP (or alternative diagnoses) should be considered when 1:
- Presenting > 30
- Unilateral symptoms
- Asymmetrical pulses
- Extant/suspected rheumatological condition
- Digital ulceration and gangrene
- Primary pernio (chilblains)
- Livedo reticularis
- Chillblain lupus
- Thoracic outlet obstruction
- Complex regional pain syndrome
Other vascular (may also cause secondary RP):
- Thromboangiitis obliterans
- Thromboembolic disease
Primary RP is a clinical diagnosis but patients may choose to pursue further workup to assess the likelihood of a predisposing condition – this can be accomplished on an outpatient basis in isolated, mild disease.
If secondary RP or an alternative diagnosis is suspected, workup should be guided by the underlying condition in question. Positive ANA and abnormal nailfold capillary pattern at capillaroscoy have positive predictive values of 30% and 50% respectively for secondary RP 3.
- Conservative e.g. cold avoidance, dressing appropriately for cold weather & smoking cessation
- Ca++ channel blockers are usually 1st line medical therapy: sustained release nifedipine or amlodipine
- ARBs, ACEIs, SSRIs and phosphodiesterase inhibitors may be considered by a specialist
Severe disease/secondary RP
- Aim to ↓ risk of vascular injury
- IV prostanoid may be warranted 6