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Raynaud's phenomenon - Management
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When should I refer someone with Raynaud's phenomenon?
- Admit immediately if the person has severe ischaemia of one or more digits.
- Refer people with Raynaud's phenomenon if:
- The diagnosis is in doubt.
- An underlying cause for the Raynaud's phenomenon is suspected, unless this is a drug which can be safely stopped.
- Most causes will be due to connective tissue disorders and referral to a rheumatologist would be appropriate.
- If an occupational cause is suspected, refer to a occupational medicine specialist, if available.
- They are a child 12 years of age or less.
- The symptoms are poorly controlled despite appropriate treatment.
Basis for recommendation
Recommendations are made on the basis of limited expert opinion, feasibility, and what CKS considers to be good clinical practice.
- An update for GPs written by an expert on behalf of the Raynaud's and Scleroderma Association states that [Raynaud's & Scleroderma Association, 2008]:
- If secondary Raynaud's phenomenon is suspected, referral to a specialist centre should be made 'if necessary'.
- 'Investigations can be arranged by the GP, or a referral can be made to a specialist'.
- Treatments for severe Raynaud's and digital ischaemia can only feasibly be given in secondary care.
- Intravenous iloprost (a prostaglandin) reduces the frequency and severity of attacks compared with placebo in people with Raynaud's phenomenon secondary to systemic sclerosis [Herrick, 2008a]. It may also be used for acute severe ischaemia with threatened digital loss [Pope, 2007b].
- Bosentan (a dual endothelin-1 receptor antagonist) may reduce new digital ulcer formation compared with placebo in people with Raynaud's phenomenon secondary to systemic sclerosis (and with previous digital ulcers in the last 12 months) [Herrick, 2008a], but should only be used under specialist supervision [BNF 56, 2008].
- Surgical interventions include:
- Digital (palmar) sympathectomy although its role has not been fully established [Herrick, 2008c].
- Stellate ganglion blocks, lumbar sympathetic blocks, and local or regional sympathectomy may be used for acute severe ischaemia with threatened digital loss [Pope, 2007b].
- Debridement of infected or necrotic tissue [Herrick, 2008c].
How should I manage someone with Raynaud's phenomenon?
- If a drug may be causing or exacerbating the Raynaud's phenomenon, review the need for it and, if possible, stop it.
- Consider whether admission or referral to secondary care is necessary, or whether it is appropriate to treat in primary care.
- Advise the following lifestyle measures:
- Keep the whole body (including the hands and feet) warm.
- Do not allow the hands and feet to get cold.
- Wear gloves and warm footwear in cold environments.
- Consider using hand and foot warming devices (contact the Raynaud's & Scleroderma Association for details).
- Avoid or stop smoking — see the CKS topic on Smoking cessation.
- Minimize stress if this is a trigger.
- Exercise regularly.
- If lifestyle measures fail, offer a trial of nifedipine as prophylaxis (the duration of which should be determined by the frequency and severity of attacks).
- Prescribe either:
- An immediate-release preparation (licensed use, but is associated with adverse effects): initially 5 mg three times daily, adjusted according to response up to 20 mg three times daily, or
- A modified-release preparation (off-licence use but may have fewer adverse effects): initially 20 mg once daily, adjusted according to response up to 60 mg once daily. See Prescriptions for more information.
- Advise that up to three-quarters of people have adverse effects, such as oedema, palpitations, headache, flushing, or dizziness.
- If nifedipine is not tolerated, consider prescribing another calcium-channel blocker, such as nicardipine, amlodipine, or felodipine (off-licence use).
- Intermittent prophylactic use (in cold weather or when participating in outdoor winter activities) may be sufficient.
- In people with primary Raynaud's phenomenon, consider periodically stopping treatment as the disease may go into remission.
- If the above measures fail and symptoms are frequent or severe, consider referral to a rheumatologist.
- For information about treatments that are not recommended for primary Raynaud's phenomenon and treatments that are not recommended in primary care for secondary Raynaud's phenomenon, see Additional information.
Additional information
- There is either evidence of no benefit or insufficient evidence to recommend the use of the following treatments for primary Raynaud's phenomenon:
- Drugs:
- Other peripheral vasodilators, including naftidofuryl oxalate, moxisylyte, buflomedil, and inositol nicotinate.
- Angiotensin-converting enzyme (ACE) inhibitors and angiotensin-II receptor antagonists.
- Phosphodiesterase inhibitors.
- Prazosin (an alpha receptor blocker).
- Complementary and alternative treatments:
- Thermal biofeedback.
- Chinese acupuncture.
- Evening primrose oil.
- Ginkgo biloba.
- The following treatments are not recommended for use in primary care for secondary Raynaud's phenomenon because of insufficient evidence or lack of feasibility:
- Drugs:
- Naftidofuryl oxalate.
- ACE inhibitors and angiotensin-II receptor antagonists.
- Alpha-receptor blockers.
- Anti-thrombotics/inhibitors of platelet aggregation.
- Glyceryl trinitrate (transdermal).
- Moxisylyte.
- Inositol nicotinate.
- Phosphodiesterase inhibitors.
- Serotonin-reuptake inhibitors.
- Oral prostaglandins.
- Bosentan (on grounds of cost and because it should be used under specialist supervision).
- Complementary and alternative treatments:
- Biofeedback.
- Relaxation therapy.
Basis for recommendation
Lifestyle measures
- In the absence of good evidence from randomized, controlled trials (RCTs) on the efficacy of the recommended lifestyle measures, recommendations are made on the basis of expert opinion in review articles [Kaufman and All, 1996; Landry et al, 1996; Waller, 1997; Wigley, 2002; Bakst et al, 2008]. Expert external reviewers remarked that, in their experience, a drop in core temperature, not just temperature in the hands or feet, can precipitate an attack.
Nifedipine for primary Raynaud's phenomenon
- The recommendation to offer a trial of nifedipine for primary Raynaud's phenomenon is based on limited evidence from a systematic review (search date: April 2003) [Thompson and Pope, 2005] and a BMJ Clinical Evidence review (search date: October 2006) [Pope, 2007a] that nifedipine reduces the frequency and severity of primary Raynaud's attacks when compared with placebo, but is associated with high rates of adverse effects.
Nifedipine for secondary Raynaud's phenomenon
- The recommendation to offer a trial of nifedipine for secondary Raynaud's phenomenon is based on limited evidence from a BMJ Clinical Evidence review (search date: May 2007) that nifedipine reduces the frequency and severity of attacks compared with placebo in people with Raynaud's phenomenon secondary to systemic sclerosis, but is associated with high rates of adverse effects [Herrick, 2008a].
Other recommendations on nifedipine prescribing
- Doses and prescriptions are recommended on the basis of recommendations from the British National Formulary, convenience, and cost (at the time of publication) [BNF 56, 2008].
- Intermittent use is advocated on the basis of limited expert opinion [Pope, 2007b].
- The recommendation to periodically stop treatment is based on study findings that remission (defined as either no attacks for two cold seasons, or 12 months without symptoms) occurs in between 3% and 33% of people with primary Raynaud's phenomenon after 7–14 years [Suter et al, 2005; Carpentier et al, 2006; Hirschl et al, 2006].
- The recommendation to consider prescribing another calcium-channel blocker if nifedipine is not tolerated is based on expert opinion [Pope, 2007b] in the absence of published evidence of efficacy.
Treatments not recommended for primary Raynaud's phenomenon
- There is no good evidence to recommend the use of drugs other than calcium-channel blockers for the treatment of primary Raynaud's phenomenon. This is on the basis of a Cochrane systematic review of oral vasodilators for primary Raynaud's phenomenon (which excluded calcium-channel blockers) [Vinjar and Stewart, 2008]; a BMJ Clinical Evidence review [Pope, 2007a]; literature reviews of phosphodiesterase inhibitors, angiotensin-converting enzyme (ACE) inhibitors, and angiotensin-receptor blockers for Raynaud's phenomenon [Levien, 2006; Wood and Ernst, 2006]; and two controlled trials [Davinroy and Mosnier, 1993; Friedman et al, 2007].
- There is insufficient good evidence to recommend the use of thermal biofeedback, Chinese acupuncture, evening primrose oil, or Ginkgo biloba extract for the treatment of primary Raynaud's phenomenon.
Treatments not recommended for use in primary care for secondary Raynaud's phenomenon
- There is no good evidence to recommend the use in primary care of drugs other than calcium-channel blockers for the treatment of secondary Raynaud's phenomenon [Herrick, 2008a].
- Bosentan (a dual endothelin-1 receptor antagonist) may reduce new digital ulcer formation compared with placebo in people with Raynaud's phenomenon secondary to systemic sclerosis (and with previous digital ulcers in the last 12 months) [Herrick, 2008a], but should only be used under specialist supervision [BNF 56, 2008].
- There is no evidence to recommend the use of biofeedback or relaxation therapy for the treatment of secondary Raynaud's phenomenon [Herrick, 2008a].
Prescriptions
For information on contraindications, cautions, drug interactions, and adverse effects, see the electronic Medicines Compendium (eMC) (http://emc.medicines.org.uk), or the British National Formulary (BNF) (www.bnf.org).
Immediate-release nifedipine
Age from 13 years to 17 years 11 months
Nifedipine capsules: 5mg three times a day
Nifedipine 5mg capsules
Take one capsule three times a day.
Supply 84 capsules.
Nifedipine capsules: 10mg three times a day
Nifedipine 10mg capsules
Take one capsule three times a day.
Supply 84 capsules.
Nifedipine capsules: 20mg three times a day
Nifedipine 10mg capsules
Take two capsules three times a day.
Supply 168 capsules.
Age from 18 years onwards
Nifedipine capsules: 5mg three times a day
Nifedipine 5mg capsules
Take one capsule three times a day.
Supply 84 capsules.
Nifedipine capsules: 10mg three times a day
Nifedipine 10mg capsules
Take one capsule three times a day.
Supply 84 capsules.
Nifedipine capsules: 20mg three times a day
Nifedipine 10mg capsules
Take two capsules three times a day.
Supply 168 capsules.
Modified-release nifedipine 20mg once a day
Age from 13 years onwards
Nifedipine m/r tablets (Adalat LA®): 20mg once a day
Adalat LA 20 tablets
Take one tablet once a day.
Supply 28 tablets.
Modified-release nifedipine 30mg once a day
Age from 13 years onwards
Nifedipine m/r tablets (Adalat LA®): 30mg once a day
Adalat LA 30 tablets
Take one tablet once a day.
Supply 28 tablets.
Nifedipine m/r tablets (Adipine XL®): 30mg once a day
Adipine XL 30mg tablets
Take one tablet once a day.
Supply 28 tablets.
Nifedipine m/r capsules (Coracten XL®): 30mg once a day
Coracten XL 30mg capsules
Take one capsule once a day.
Supply 28 capsules.
Modified-release nifedipine 40mg once a day
Age from 13 years onwards
Nifedipine m/r tablets (Fortipine LA 40®): 40mg once a day
Fortipine LA 40 tablets
Take one tablet once a day.
Supply 30 tablets.
Modified-release nifedipine 60mg once a day
Age from 13 years onwards
Nifedipine m/r tablets (Adalat LA®): 60mg once a day
Adalat LA 60 tablets
Take one tablet once a day.
Supply 28 tablets.
Nifedipine m/r tablets (Adipine XL®): 60mg once a day
Adipine XL 60mg tablets
Take one tablet once a day.
Supply 28 tablets.
Nifedipine m/r capsules (Coracten XL®): 60mg once a day
Coracten XL 60mg capsules
Take one capsule once a day.
Supply 28 capsules.
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