- Clinical science
Raynaud phenomenon (RP) is an exaggerated vasoconstrictive response of the digital arteries and arterioles (e.g., in the fingers and/or toes) to cold or emotional stress. It is termed primary or secondary based on the underlying cause. The etiology of primary RP is poorly understood. Secondary RP, on the other hand, is caused by underlying systemic diseases (e.g., mixed connective tissue disease, vasculitides, hematologic abnormalities). Both types typically present with the sequential discoloration of fingers and/or toes from white (ischemia) to purplish-blue (hypoxia) to red (reactive hyperemia). Episodes of vasoconstriction usually end 15–20 minutes after the trigger is removed, and last no longer than an hour following adequate warming or stress reduction. Secondary RP may be accompanied by complications of underlying diseases and/or trophic disorders. Management involves the treatment of any underlying conditions, avoidance of situations that may trigger an attack, and calcium channel blockers (e.g., nifedipine, diltiazem). Rubefacients, or vasoactive agents, are indicated in severe cases.
Cold and emotional stress are common triggers of vasospastic attacks in patients with primary or secondary RP. Occurs more commonly in women.
Primary RP (also called Raynaud disease)
- Idiopathic genesis, without any identifiable organic vascular change, leading to vasospasms of the digital arteries and arterioles.
- Onset usually < 30 years of age
Secondary RP (also called Raynaud syndrome)
Organic arterial changes in the fingers (and/or toes) that lead to vasospasm, which may be caused by:
- Drugs: beta-blockers, ergotamine, bleomycin
- Occupational trauma: from handling vibrating tools, typing
- Hyperviscocity: ), , cold agglutinin disease, paraproteinemias ( ,
- Vasculitides: e.g., Buerger's disease
- Connective tissue diseases: e.g., , , ,
- Arterial disease: e.g.,
- Neurological disease: e.g., ,
- Onset usually ≥ 30 years of age
Ischemic phase (white): exposure to trigger (e.g., cold); → vasoconstriction of digital arteries and arterioles→ ischemia and pallor
- Sometimes accompanied by pin-and-needles sensation, numbness, or pain
- Hypoxic phase (blue): low oxygen supply → cyanosis
- Hyperemic phase (red): rewarming or removal of stressor leads to recovery and reperfusion → erythema
- might occur during attacks
- Ischemic phase (white): exposure to trigger (e.g., cold); → vasoconstriction of digital arteries and arterioles→ ischemia and pallor
An attack does not always involve all three phases; often, the hyperemic phase does not occur!
|Primary Raynaud phenomenon||Secondary Raynaud phenomenon|
|Clinical picture|| || |
- Most commonly the fingers and toes
- Rarely the nose, ears, nipples, and lips
- Duration: Spasms are usually reversible, typically lasting 15–20 minutes after removing the trigger, but may last for up to an hour.
Irreversible ischemia with tissue damage indicates secondary RP and requires further investigation to identify the underlying cause!
While primary RP is primarily a clinical diagnosis, additional testing is required to diagnose secondary RP; . Differentiating between the two types enables the practitioner to identify and assess the severity of any possible underlying condition.
- Assessment of patient history focuses on: onset of symptoms, triggers of attacks, time course, pattern of attacks, accompanying symptoms (pain, paresthesias), and impairment of everyday life.
- See “Etiology” and “Symptoms/Clinical findings” above.
Nailfold capillary microscopy
- Important means of distinguishing primary from secondary RP
- Primary RP: normal capillaroscopic pattern
- Secondary RP: abnormal capillaroscopic pattern.
- All values typically normal in primary RP
- In suspected secondary RP, analyses should focus on diagnosis of underlying diseases.
The general approaches to primary and secondary RP are similar. However, in cases of secondary RP, the underlying condition should also be treated.
- Avoid triggers: dampness, cold , emotional stress
- Stop smoking
- Adjust medication (discontinue drugs that may cause attacks: e.g., beta-blockers, ergotamine, oral contraceptives)
- First‑line: calcium channel blockers; the drug of choice is nifedipine administered orally
- Topical agents to increase perfusion (e.g., topical nitroglycerin)
- Off-label therapy with vasoactive agents
- Trophic disorders (rare in primary RP)
- Other systemic complications of underlying disease in secondary RP
We list the most important complications. The selection is not exhaustive.