Raynaud's Phenomenon (Guidelines)

• Onset is typically in adolescence / young adulthood and episodes do not usually result in digital ulceration.
• Often there is a family history of Raynaud’s phenomenon present.
• It is more commonly seen in females and with low body mass index.

This is where an underlying cause for Raynaud’s phenomenon can be identified. It is much less common than Primary RP.

Secondary RP should be suspected when onset is later in life e.g. age >30 years and particularly when episodes result in tissue injury.

Causes include: 

• Connective tissue disease (systemic sclerosis, SLE, dermatomyositis, mixed connective tissue disease, antiphospholipid syndrome)
• Other rheumatic diseases
• Drugs (chemotherapy, beta blockers, amphetamines, cocaine, ADHD drugs, nasal decongestants)
• Use of vibrating tools
• Paraproteinaemia
• Large vessel disease (may cause unilateral RP episodes - check upper limb pulses)
• And hypothyroidism.

It is important to check for features that may suggest connective tissue disease (e.g. nailfold infarcts, puffy fingers, skin tightening, telangiectasia, calcinosis, GORD / oesophageal dysmotility for systemic sclerosis, photosensitive rashes, malar rash, hair fall, mouth ulcers, arthralgia, myalgia etc. for SLE).

Raynaud’s phenomenon symptoms are more commonly reported in patients with fibromyalgia although this is felt to be more related to cold intolerance rather than an increased prevalence of true RP.

Investigations

Primary RP

• investigations are usually not necessary unless there are other symptoms to suggest a secondary cause. Cases can usually be managed in primary care as per guidance below.

Secondary RP

Investigations in primary care prior to referral:

• urinalysis, FBC, PV, U&E, glucose, CRP, TSH, Connective tissue screen.
• Further investigations: DNA antibodies, complement C3 and C4, CK, serum protein electrophoresis

Further Investigations in Secondary care may include:

• scleroderma blot (suspected systemic sclerosis), antiphospholipid antibodies (suspected SLE or APS), and myositis blot (suspected dermatomyositis / polymyositis), CT angiogram (suspected proximal arterial occlusion).
• Nailfold capillaroscopy may identify abnormal capillaries associated with connective tissue disease.

Non-pharmacological management:

• cold avoidance, gloves (including heated gloves)
• maintenance of core body temperature (layered clothing, warm socks, thermal underwear and vests / leggings)
• smoking cessation
• avoidance of caffeinated drinks and known precipitants
• stop aggravating drugs (e.g. beta blockers) if possible.

Pharmacological management:

May be initiated in primary care for the management of Primary Raynaud’s phenomenon.

• Calcium channel antagonists: nifedipine (preferably modified release). Initially 10mg daily, titrated as necessary to 60mg daily). (Off-label indication).
• Amlodipine (5mg to 10mg daily) and diltiazem are alternatives. (Off label indications).

If inadequate response to calcium channel antagonist or intolerant, other therapeutic options, to be discussed with secondary care prior to initiation in primary care include:

• Phosphodiesterase-5 inhibitors: e.g. sildenafil 25mg three times daily, increased if necessary to 50mg three times daily. (Off label indication).
• Angiotensin II receptor antagonist: e.g. losartan 25mg to 50mg daily. (Off label indication).
• Topical nitrate: e.g. 0.4% GTN ointment applied to affected digit or palm.
  Avoid if patient taking phosphodiesterase inhibitor due to risk of hypotension.
  Aim for nitrate-free period of at least 12 hours to avoid tolerance. (Off label indication).
• SSRI: fluoxetine 20mg daily may be considered when other options are not tolerated, particularly if hypotension has been problematic. (Off label indication).

In people with primary Raynaud’s phenomenon, consider periodically stopping treatment as the disease may go into remission.

No routine monitoring is required for primary RP cases. Referral to secondary care if complications of RP (e.g. digital ulcers) or difficult to control RP, which might suggest an underlying secondary cause.

Secondary care referral should be considered in cases where secondary RP is likely (e.g. late onset RP plus features/signs of a connective tissue disease or positive autoantibodies) and particularly when there is evidence of tissue damage.

Referral is via SCI gateway and to include the test detailed as above.

Other pharmacological options considered by the specialist include:

Iloprost (hospital only)

• Reserved for patients with severe disease and ischaemic complications. Given by intravenous infusion.
  Currently capacity at HRU infusion suite limited to maximum of 3 consecutive days.
• Wound management and antibiotic therapy may also be required in cases of digital ulceration and imaging to exclude deep infection.
• For administration see: Iloprost in adults with severe Raynaud’s phenomenon (Guidelines)

Botulinum toxin type A injections

• Can also be considered, especially in patients with digital ulcerations, if other pharmacological treatments not well tolerated or not effective.

Bosentan (specialist initiation only)

• May be considered in patients with systemic sclerosis with digital ulceration (to reduce number of new digital ulcers).
  62.5mg twice daily for 4 weeks then increased to 125mg twice daily.