From the Guidelines
The workup of Raynaud's phenomenon should begin with a thorough history and physical examination to distinguish between primary (idiopathic) and secondary (associated with underlying conditions) forms, and first-line pharmacological treatment typically involves calcium channel blockers like nifedipine (30-60 mg daily) or amlodipine (5-10 mg daily) 1. The initial evaluation should include complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, C-reactive protein, antinuclear antibody, and nailfold capillaroscopy. If secondary Raynaud's is suspected, additional tests may include specific autoantibodies (anti-centromere, anti-Scl-70), chest X-ray, pulmonary function tests, and echocardiogram to evaluate for associated connective tissue diseases like systemic sclerosis 1. Management includes lifestyle modifications such as:
- avoiding cold exposure
- smoking cessation
- stress reduction
- wearing proper (warm) clothing in cold conditions, such as a coat, mittens, hat, dry insulated footwear and hand and/or foot warmers 1 For patients with inadequate response, phosphodiesterase-5 inhibitors (sildenafil 20 mg three times daily) may be considered, as they have been shown to improve Raynaud’s condition score, daily frequency of RP attacks, and daily duration of RP attacks in minutes 1. Severe cases with digital ulcers or threatened tissue loss require urgent referral to specialists and may need intravenous prostacyclins, such as iloprost 1. The pathophysiology involves abnormal vasoconstriction of digital arteries in response to cold or emotional stress, with secondary Raynaud's often showing structural vascular abnormalities and immune dysregulation that requires targeted treatment of the underlying condition. Some key points to consider in the management of Raynaud's phenomenon include:
- Bosentan should be considered for reduction of number of new digital ulcers in systemic sclerosis 1
- Dihydropyridine-type calcium antagonists, usually oral nifedipine, should be used as first-line therapy for systemic sclerosis-related Raynaud's phenomenon 1
- Topical nitrates, such as nitroglycerin or glyceryl trinitrate, may be considered for treatment of Raynaud's phenomenon, but may have limiting adverse effects such as headache 1
From the Research
Definition and Prevalence of Raynaud Phenomenon
- Raynaud phenomenon (RP) is defined as recurrent, reversible episodes of vasospasm involving peripheral small vessels, typically in the fingers and toes 2.
- The prevalence of Raynaud's phenomenon in the general population has been reported to be approximately 5% 2, 3, 4, 5.
Primary and Secondary Raynaud Phenomenon
- Primary (idiopathic) RP is common and usually benign, occurring in about 5% of the general population 2.
- Secondary RP accounts for 10% to 20% of all RP cases and may be associated with complications such as tissue loss, ulcers, and gangrene 2.
- Systemic sclerosis (SSc) or other connective tissue diseases are the main underlying conditions for secondary RP 2, 3, 4.
Diagnosis and Workup
- A detailed clinical history and careful physical examination may be helpful in identifying the cause of RP 2.
- Routine investigations include a full blood count, measurement of erythrocyte sedimentation rate, C‑reactive protein, antinuclear antibody levels, biochemical profile, thyroid function tests, protein electrophoresis, chest X‑ray, and nailfold capillaroscopy 2.
- Capillaroscopy can facilitate a very early diagnosis of SSc 2.
- Doppler ultrasound is recommended to evaluate the risk of pathologies in large to medium-sized arteries 2.
Management and Treatment
- Lifestyle modifications may be sufficient to control primary RP, but some patients, and most with secondary RP, require pharmacologic treatment 2.
- Several medications are proposed to manage RP and its complications, such as calcium channel blockers, phosphodiesterase type 5 inhibitors, intravenous prostanoids, and topical nitrates 2, 5.
- Non-pharmacological management, including patient education and lifestyle adaptations, is first-line treatment for RP 3, 4, 5.
- Surgical intervention is sometimes required for refractory RP and tissue ischaemia 3, 4.