Raynaud's Phenomenon

Overview

Clinical Knowledge Summaries has a useful summary with advice on investigations to consider and management in primary care:

(1) Raynaud's phenomenon | Health topics A to Z | CKS | NICE

Clinical Features

Raynaud's phenomenon is episodic vasospasm of the arteries or arterioles in the extremities (usually the digits) which leads to colour change including pallor, followed by cyanosis and/or rubor.

Episodes are typically precipitated by cold exposure and/or emotional distress. Can affect fingers, toes, nose, ears and areolar tissues.

Classification

Raynaud’s phenomenon can be classified as:

• Primary Raynaud's phenomenon (80–90% of cases) which occurs without an associated underlying condition.
• Secondary Raynaud's phenomenon (10–20% of cases) which occurs in association with an underlying condition (often a connective tissue disorder such as scleroderma or systemic lupus erythematosus). (1)

Who to Refer

Referral is recommended for:

• All children aged 12 years or less with features of Raynaud's phenomenon - See the Paediatric Rheumatology page.
• All people with suspected secondary Raynaud's phenomenon. (1) - see further advice below from local rheumatologists:

Secondary causes

1. Late onset Raynaud’s (>30 years) is typically secondary in nature and should be investigated further in all cases to determine cause.
2. Raynaud’s with positive ANA (sometimes with puffy fingers) should prompt referral to rheumatology
3. Unilateral symptoms suggests a secondary cause (usually related to proximal obstruction to large vessels e.g. thoracic outlet)
4. Isolated involvement of toes (without finger involvement) should prompt Ix for secondary macrovascular cause and ABPIs (plus onward referral to vascular surgery if required)
5. Any digital ischaemic lesions (ulceration, localised necrosis) should lead to onward referral to exclude secondary cause.

Red Flags

If severe ischaemia of one or more digit is suspected please discuss immediately with on call vascular surgery team - see Peripheral Arterial Disease page for details.

What to do before referral

History (including medication) and examination can help to direct investigations and exclude secondary (see Causes of, or diseases associated with Raynaud's phenomenon).

Bloods in primary care:

• FBC
• TSH
• CRP -  may be normal unless significant necrosis/infection or co-existent inflammatory features. (ESR is usually raised but not routinely offered in local laboratories).
• Anti-nuclear antibodies (ANA) - (search under Connective Tissue Disease on UHBW ICE and Anti-nuclear on NBT ICE). If the test is negative and the patient is otherwise asymptomatic, there is a low clinical probability of the patient having an underlying rheumatological condition. A positive ANA occurs in approximately 5% of the adult population and alone has poor diagnostic value in the absence of clinical features of autoimmune rheumatic disease.
• Other bloods may also be considered depending on suspected cause.

Other investigations to consider:

• ABPIs -  if patient has vascular risk factors
• CXR - to look for cervical rib if there are unilateral features or possible thoracic outlet features.

Referral

Most patients with primary Raynaud's phenomenon can be managed in primary care with reassurance and advice. A trial of medication can also be appropriate in primary care if symptoms are more severe/frequent and  secondary causes have been excluded.( Management | Raynaud's phenomenon | CKS | NICE )

Referral to an appropriate specialist may be indicated if Secondary Raynauds Phenonomen is suspected following initial investigation in primary care.

Rheumatology Referral

Consider rheumatology referral via eRS or advice and guidance if the history/ examination or investigations suggest a rheumatological cause. 

There is now a Raynaud’s clinic at NBT (led by John Pauling) accepting referrals for suspected secondary Raynaud’s with diagnostic microvascular imaging studies (nailfold capillaroscopy).

Vascular Referral

Consider a vascular referral via eRS (RAS) for patients with isolated involvement of toes (without finger involvement) for ABPIs and vascular surgery review if required - see Peripheral Arterial Disease page for details.

Resources

Patient information on Raynaud's phenomenon

• NHS Choices Raynaud’s.
• Scleroderma and Raynaud’s UK.

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