Last reviewed: November 2017
Last updated: December  2017

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • Raynaud's phenomenon (RP)
  • dysphagia
  • GORD
  • dilated nailbed capillaries
  • bright shiny skin of hands, feet
  • symmetrical swelling, tight fingers
  • prayer sign
  • claw hand deformities
  • ulcers on fingers
  • flexion contracture of hands
  • carpal tunnel syndrome
  • calcinosis
  • large well-demarcated telangiectasia

Other diagnostic factors

  • skin pigmentation changes
  • FHx of connective tissue disease

Risk factors

  • silica dust exposure
  • FHx of scleroderma
  • polyvinylchlorides

Diagnostic investigations

1st investigations to order

  • FBC
  • serum creatinine
  • serum ANA
  • serum extractable nuclear antigens (ENA)
  • serum ESR
  • echocardiogram
  • pulmonary function tests (PFTs)
Full details

Investigations to consider

  • peripheral blood smear
  • anti-centromere antibody
  • oesophageal manometry
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Janet E. Pope

Professor of Medicine

Division of Rheumatology

Department of Medicine

Schulich School of Medicine and Dentistry

University of Western Ontario

Head

Division of Rheumatology

St. Joseph's Health Care

London

Ontario

Canada

Disclosures

JEP has consulted for and/or performed research trials with AbbVie, Actelion, Amgen, BMS, Celgene, GSK, Hospira, Janssen, Lilly, Merck, Novartis, Pfizer, Roche, Sandofi, and United Chemicals Belgium. JEP is an author of several references cited in this monograph.

Peer reviewers VIEW ALL

Professor of Medicine

Georgetown University

Washington

DC

Disclosures

VS has received research funding, consulting fees, and honorarium from Actelion and Gilead, which could result in competing interests. VS is an author of a number of references cited in this monograph.

Emeritus Professor

Department of Rheumatology

Lund University Hospital

Lund

Sweden

Disclosures

FW is an author of a reference cited in this monograph.

Charité-Universitätsmedizin Berlin

Medizinische Klinik mit Schwerpunkt

Rheumatologie und Klinische Immunologie

Charitéplatz

Berlin

Germany

Disclosures

GR is a consultant for Encysive, Actelion, Bayer, GSK, Roche, and other companies, and received lecture fees from all of these companies involved in systemic scleroderma. GR is an author of a reference cited in this monograph.

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