QuestionSBA
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Apixaban is an anticoagulant (in the DOAC category) that is often used in the treatment of deep vein thrombosis and in patients who are pro-coagulant. It is not currently used in the treatment of Raynaud's phenomenon
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Apixaban
QuestionChoice
This patient most likely has Raynaud's phenomenon as she is experiencing paroxysmal digital ischaemia when exposed to the cold. It is often associated with underlying rheumatological conditions, including systemic sclerosis, rheumatoid arthritis and systemic lupus erythematosus (SLE). Treatment is mostly trigger management - keeping fingers warm, smoking cessation and avoiding stress - however in some cases, nifedipine may help symptoms
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Nifedipine
QuestionChoice
Warfarin is an anticoagulant that is sometimes used in patients who are pro-coagulant or who have venous thrombosis, particularly if they have failed treatment with a direct oral anticoagulant (DOAC). It is also used in patients with metal heart valves. It is not of benefit in this patient's case
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Warfarin
QuestionChoice
Epoprostenol is also known as prostaglandin I2 and is an effective vasodilator. It is sometimes used to treat severe attacks of Raynaud's, e.g. where there are concerns about digital gangrene or the development of digital necrosis. It is a rescue therapy and is not used routinely. Relapse is common after infusion, and some patients with severe disease require regular infusions, particularly in the winter months
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Epoprostenol
QuestionChoice
Aspirin is an antiplatelet medication often used in peripheral vascular disease. While this patient has digital ischaemia, it is paroxysmal; therefore, aspirin is not the treatment of choice
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Aspirin
QuestionComment
how does everyone know this lol
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subscriber
hiney
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# Summary

Raynaud's phenomenon refers to episodes of vasospasm of small arteries and arterioles, which leads to reduced blood flow to the extremities, usually the fingers and toes. This classically manifests with the digits going pale, then cyanosed, then erythematous (with this final stage often being uncomfortable). Triggers include cold exposure and emotional stress. The majority of cases are classified as primary, however around 10% of cases are associated with underlying disease such as systemic sclerosis or polycythaemia. Diagnosis is clinical; however underlying causes should be screened for with a full blood count, ESR and antinuclear antibodies. Other investigations should be carried out if an underlying cause is suspected. Management involves lifestyle changes such as wearing gloves and warm footwear, smoking cessation and reviewing medications that may exacerbate Raynaud's. If conservative measures are ineffective, medical treatment with nifedipine can be considered. Patients with suspected secondary Raynaud's phenomenon should be referred to rheumatology. 

# Definition

Raynaud's phenomenon is a condition where arteries or arterioles constrict, decreasing blood flow to the affected areas. Attacks are usually triggered by cold exposure, with other triggers including emotional stress, vibration injury, smoking and medications (e.g. beta-blockers). The fingers and toes are the most commonly affected areas. 

# Epidemiology

- Primary Raynaud's phenomenon is common and affects around 5% of people
- It is more common in women and in people living in colder climates
- Other risk factors include:
- Family history
- Smoking
- Migraine
- Secondary Raynaud's phenomenon is much less common, making up 10-20% of cases
- Patients with secondary Raynaud's phenomenon tend to be older than patients with primary disease (suspect if symptoms start over the age of 30)

# Classification

**Primary Raynaud's phenomenon** (PRP, also known as "idiopathic" Raynaud's) is not associated with an underlying cause

- It typically develops between the ages of 10 and 30
- It is usually a benign condition which may improve with age
- Although some patients may experience significant discomfort with severe primary Raynaud's, complications such as ischaemia or ulceration are not seen

**Secondary Raynaud's phenomenon** (SRP) may be associated with a variety of pathologies including:

- Rheumatological conditions e.g. systemic sclerosis, systemic lupus erythematosus, dermatomyositis, rheumatoid arthritis and Sjogren's syndrome
- Haematological conditions e.g. polycythaemia, protein C/S/antithrombin III deficiency, paraproteinaemia, Factor V Leiden
- Medications e.g. beta-blockers, methylphenidate, some chemotherapy agents and interferons
- Recreational drugs e.g. cocaine, amphetamines
- Occupational causes e.g. vinyl chloride exposure, hand-arm vibration syndrome (due to long-term use of tools such as chainsaws or grinders)
- Extrinsic compression of vessels e.g. carpal tunnel syndrome
- Endocrine conditions e.g. hypothyroidism, carcinoid syndrome and phaeochromocytoma

Symptoms of secondary Raynaud's phenomenon are usually more severe than in PRP, especially in cases secondary to a connective tissue disorder.

Features that should raise suspicion of SRP include:

- Onset after age 30
- Asymmetrical digit involvement
- Intense and painful episodes
- Digital ulcers, gangrene or ischaemia
- Abnormal nail fold capillaroscopy
- Clinical features suggestive of underlying disease (e.g. sclerodactyly)
- Positive antinuclear antibodies

# Signs and Symptoms 

- Typically the tips of the digits go pale - this may spread proximally and is clearly demarcated
- They may then become blue and cyanosed
- The affected digits may be numb or painful
- This is often followed by a hyperaemic phase where the affected areas become erythematous with associated discomfort and paraesthesias
- The thumb is usually spared
- Other areas that may be affected include:
- Tip of the nose
- Ear lobes
- Nipples
- Tongue

[lightgallery] 

Especially in PRP, there may be no signs on examination unless the patient is currently experiencing an attack. 

Signs in SRP may include:

- Abnormal nailfold capillaries (examine with a dermatoscope)
- Asymmetrical peripheral pulses or blood pressure between arms
- Ulceration, ischaemia or necrosis of digits
- Scarring
- Secondary infection

# Differential Diagnosis

- **Frostbite** - occurs when tissues are frozen, causing microvascular occlusion and ischaemia. Skin is cold and numb, and may be discoloured and purple with blistering and necrosis in more severe cases.
- **Chilblains** - localised inflammatory lesions caused by cold exposure; itchy, red-purple swellings form that may blister and ulcerate.
- **Acrocyanosis** - persistent painless cyanosis of the extremities that is exacerbated by cold exposure, usually associated with clamminess and hyperhidrosis
- **Erythromelalgia** - vasodilatation of small vessels causes burning pain, warmth and erythema of the hands and feet which may be continuous or episodic; often triggered by heat or exercise
- **Livedo reticularis** - red-blue mottling of the skin that in physiological cases is triggered by cold weather (in pathological cases the rash is persistent)
- **Vascular occlusion** e.g. acute cholesterol embolism may cause blue discolouration of the toes however this is not episodic; there is often ischaemia of other organs and a systemic inflammatory response

# Investigations

- Patients should all have basic bloods for the following:
- **Full blood count** - looking for polycythaemia, a thrombocytosis may be seen in inflammatory states
- **Erythrocyte sedimentation rate** (ESR) - raised in inflammatory conditions
- **Antinuclear antibodies** (ANA) - positive in around 10% of the general population, associated with some causes of SRP such as systemic lupus erythematosus and scleroderma 

Other investigations should be targeted if there are signs and symptoms of another underlying cause, e.g. thyroid function tests, testing for prothrombotic conditions, chest X-ray looking for a cervical rib

Patients with suspected SRP should be referred for nailfold capillaroscopy to examine these for abnormalities (such as widened capillaries and microhaemorrhages in systemic sclerosis)

# Management

**Conservative:**

- Lifestyle advice and education:
- The whole body should be kept warm, with gloves and warm footwear worn in cold weather
- Hand and foot warming devices may be useful
- Regular exercise
- Smoking cessation
- Address emotional stress if this is a trigger
- Review medications for triggers (such as beta-blockers) and consider stopping or switching to an alternative if possible
- Occupational health referral for patients whose Raynaud's phenomenon may be work-related (e.g. secondary to vibrating tools)
- Patients with suspected secondary Raynaud's phenomenon should be referred to rheumatology, as well as patients with severe or treatment-resistant primary Raynaud's
- Patients with severe ischaemia of digits or other significant complications require emergency hospital admission

**Medical:**

- Nifedipine (a calcium channel blocker) as the first-line prophylactic treatment
- If this is not tolerated (common side-effects being headaches, flushing, ankle swelling, palpitations and dizziness), an alternative is amlodipine
- Patients may take this regularly or intermittently e.g. when the weather is cold or when doing outdoor activities
- Secondary care options for patients with severe complications include intravenous prostaglandins (e.g. iloprost), sildenafil and bosentan (an endothelin receptor antagonist)

**Interventional:**

- Rarely, some patients with severe disease may be offered a sympathectomy, where sympathetic nerves that cause vasoconstriction are resected surgically
- Botox injections to block the sympathetic nerves in the hands and/or feet are also an option

# Prognosis

- Primary Raynaud's syndrome is typically benign and may improve over time
- Some patients enter a state of remission where they no longer experience episodes
- However some patients who have severe symptoms may find their quality of life is affected by discomfort and functional impairment
- Secondary Raynaud's syndrome is in general more severe and may lead to complications including:
- Digital ischaemia, with pitting, ulceration or gangrene
- Tissue loss and scarring
- Poor wound healing and secondary infection
- The prognosis for secondary Raynaud's syndrome is variable depending on the underlying cause and whether this is avoidable or treatable

# NICE Guidelines

[NICE CKS - Raynaud's Phenomenon](https://cks.nice.org.uk/topics/raynauds-phenomenon/)

# References

[Patient UK - Raynaud's Phenomenon](https://patient.info/doctor/raynauds-phenomenon-pro)
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The typical appearance of raynaud's syndrome.
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Nifedipine, a calcium channel blocker, is commonly used to treat Raynaud's disease by relaxing blood vessels and improving blood flow, reducing the frequency and severity of episodes of vasoconstriction and color changes in the fingers.
A 48-year-old female presents to General Practice complaining of cold, painful fingers. She reports that her fingers go blue after being outside (particularly if she forgets her gloves) but that they regain their colour when she comes inside. Her only past medical history is significant for rheumatoid arthritis, for which she takes methotrexate.

What medication should you consider starting?
a