Summary
Definition
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
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
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
Diagnostic tests
1st tests to order
- clinical diagnosis
- antinuclear antibody
- CBC
- erythrocyte sedimentation rate
- C-reactive protein
- urinalysis
Tests to consider
- capillaroscopy
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
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
GA
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
UK
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
UK
Disclosures
BG declares that she has no competing interests.
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- Normal response to cold
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