Last reviewed: May 2019
Last updated: June  2018

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
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
  • auto-amputation
Full details

Risk factors

  • female
  • FHx
  • connective tissue disease
  • vibration injury
  • Buerger's disease
  • prolonged cold exposure/frostbite
  • colder climate
  • smoking
  • ischaemia
  • migraine
  • glaucoma
Full details

Diagnostic investigations

1st investigations to order

  • clinical diagnosis
  • ANA
  • FBC
  • ESR
  • creatinine
  • urinalysis
Full details

Treatment algorithm

ACUTE

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

ONGOING

primary or mild secondary RP

Contributors

Authors VIEW ALL

Janet Pope

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 consulted for Actelion, Bayer, Merck, and Pfizer.

Peer reviewers VIEW ALL

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 monograph.

Consultant Rheumatologist

Department of Rheumatology

Freeman Hospital

Newcastle upon Tyne

UK

Disclosures

BG declares that she has no competing interests.

Intern

Veterans Administration Hospital

Minneapolis

MN

Disclosures

KO declares that he has no competing interests.

Associate Professor

Department of Medicine

Mayo Clinic

Rochester

MN

Disclosures

TO declares that he has no competing interests.

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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