Summary
Definition
History and exam
Key diagnostic factors
- presence of risk factors
- digit pain/discomfort
- digital paraesthesia
- 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
- auto-amputation
Risk factors
- female
- family history
- connective tissue disease
- vibration injury
- Buerger's disease
- prolonged cold exposure/frostbite
- colder climate
- smoking
- ischaemia
- migraine
- glaucoma
Diagnostic investigations
1st investigations to order
- clinical diagnosis
- antinuclear antibody
- FBC
- erythrocyte sedimentation rate
- creatinine
- urinalysis
Treatment algorithm
severe secondary RP: critical ischaemia 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 attended advisory boards for Actelion, the manufacturer of bosentan. JP is an author of a number of references cited in this topic.
Peer reviewers
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.
Kim Olson, MD
Intern
Veterans Administration Hospital
Minneapolis
MN
Disclosures
KO declares that he has no competing interests.
Thomas Osborn, MD
Associate Professor
Department of Medicine
Mayo Clinic
Rochester
MN
Disclosures
TO declares that he has no competing interests.
Philip Seo, MD, MHS
Assistant Professor of Medicine
Division of Rheumatology
Johns Hopkins University School of Medicine
Co-Director
Johns Hopkins Vasculitis Center
Baltimore
MD
Disclosures
PS declares that he has no competing interests.
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