Raynaud phenomenon

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Last reviewed: 5 Oct 2024

Last updated: 04 Oct 2024

Raynaud phenomenon (RP) is common, affecting between 3% and 5% of the population.

The diagnosis is made clinically: digits turn white (pallor) then blue with deoxygenation and/or red with reperfusion; pallor is well demarcated.

Primary RP often needs no pharmacologic treatment. Keeping warm, smoking cessation, regular exercise, and avoiding stress are recommended. Pharmacologic treatment can be considered in people with mild disease who do not respond to lifestyle measures. Potential treatment options include calcium-channel blockers, angiotensin-II receptor antagonists, selective serotonin-reuptake inhibitors (SSRIs), topical nitrates, alpha-blockers, and ACE inhibitors.

Secondary RP can be severe, especially when associated with systemic sclerosis (SSc). Other underlying conditions, exposures, or drugs can also cause secondary RP. In severe secondary RP, calcium-channel blockers, phosphodiesterase-5 inhibitors, prostacyclins, atorvastatin, and bosentan are potential treatment options, with only the most severe cases warranting surgical intervention.

Complications in secondary RP include severe digital ischemia, gangrene, digital ulcers, and infection.

Pain relief and wound care of digital ulcers may be important adjunctive treatments.

Definition

Raynaud phenomenon (RP) is characterized by vasospasm that causes digits to change color to white (pallor) from lack of blood flow, usually brought on by cold temperatures. Affected areas subsequently turn blue due to deoxygenation and/or red due to reperfusion. Both primary and secondary RP can be painful; however, secondary RP can lead to complications from prolonged periods of ischemia.

History and exam

Key diagnostic factors

digit pain/discomfort
digital paresthesia
pallor of digits
red and/or blue discoloration of digits
dilated capillaries at nailbeds
well-defined discoloration
magnification of nailbeds

Full details

Other diagnostic factors

heartburn
dysphagia
puffy hands
tight skin
arthralgia
photosensitivity
oral/nasal ulcers
alopecia
butterfly rash
sclerodactyly
telangiectasia
pleuritic chest pain
digital ulcers
digital pits
digital tuft resorption
gangrene of fingertip/finger
raised painful red lesions on finger tips
autoamputation

Full details

Risk factors

female
family history
connective tissue disease
use of certain drugs
vibration injury
Buerger disease
prolonged cold exposure/frostbite
colder climate
smoking
ischemia
migraine
glaucoma

Full details

Diagnostic tests

1st tests to order

clinical diagnosis
antinuclear antibody
CBC
erythrocyte sedimentation rate
C-reactive protein
urinalysis

Full details

Tests to consider

capillaroscopy

Full details

Treatment algorithm

ACUTE

severe secondary RP: critical ischemia with digital ulcers or threatened digital loss

ONGOING

primary or mild secondary RP

Contributors

Authors

Janet Pope, MD, MPH, FRCPC

Professor of Medicine

Division of Rheumatology

Department of Medicine

University of Western Ontario

Schulich School of Medicine and Dentistry

Head

Division of Rheumatology

St. Joseph's Health Care

London

Ontario

Canada

Disclosures

JP has been reimbursed for consulting with several pharmaceutical manufacturers unrelated to Rayaud phenomenon. JP’s institution receives research grants from BMS, Janssen, and Seattle Genetics. She has acted as a consultant for AbbVie, Amgen, AstraZeneca, BI, BMS, Celltrion, EMERALD, Frensenius Kabi, GSK, Janssen, JMP, Lilly, Mallinckrodt Pharmaceuticals, Mitsubishi Tanabe Pharma, Novartis, Organon, Pfizer, Sandoz, Samsung, and Viatris. JP is a speaker and advisory board member for AbbVie, Amgen, AstraZeneca, BI, BMS, Frensenius Kabi, GSK, Janssen, JMP, Lilly, Novartis, Organon, Pfizer, Sandoz, UCB, and Viatris. She receives no patents or royalties. JP is an author of a number of references cited in this topic.

Peer reviewers

Elena Schiopu, MD

Professor of Medicine

Rheumatologist

Medical College of Georgia at Augusta University

Charlie Norwood Veterans Affairs Medical Center

Augusta

Disclosures

ES declares that she has no competing interests.

Ariane L. Herrick, MD FRCP

Reader in Rheumatology and Consultant Rheumatologist

Musculoskeletal Research Group

University of Manchester

Manchester

Disclosures

ALH has been a paid speaker for Actelion; a consultant for Actelion and Pfizer; and an investigator in studies sponsored by Actelion, Mediquest, and United Therapeutics. ALH is an author of a number of references cited in this topic.

Bridget Griffiths, MB ChB, MD, MRCP(UK)

Consultant Rheumatologist

Department of Rheumatology

Freeman Hospital

Newcastle upon Tyne

Disclosures

BG declares that she has no competing interests.

Differentials
- Normal response to cold
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- Chilblains (perniosis)
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Guidelines
- The 2024 British Society for Rheumatology guideline for management of systemic sclerosis
- EULAR recommendations for the non-pharmacological management of systemic lupus erythematosus and systemic sclerosis
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Patient information
Quitting smoking
Raynaud phenomenon
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Raynaud phenomenon

Summary

Definition

History and exam

Key diagnostic factors

Other diagnostic factors

Risk factors

Diagnostic tests

1st tests to order

Tests to consider

Treatment algorithm

severe secondary RP: critical ischemia with digital ulcers or threatened digital loss

primary or mild secondary RP

Contributors

Authors

Janet Pope, MD, MPH, FRCPC

Disclosures

Peer reviewers

Elena Schiopu, MD

Disclosures

Ariane L. Herrick, MD FRCP

Disclosures

Bridget Griffiths, MB ChB, MD, MRCP(UK)

Disclosures

Differentials

Guidelines

Patient information

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