Last reviewed: July 2020
Last updated: March  2018



History and exam

Key diagnostic factors

  • presence of risk factors
  • Raynaud's phenomenon
  • digital pits or ulcers
  • swelling of the hands and feet
  • skin thickening
  • loss of function of hands
  • sclerodactyly
  • heartburn, reflux, and dysphagia
  • bloating
  • faecal incontinence
  • arthralgias and myalgias
  • abnormal nail-fold capillaroscopy
  • telangiectasia
  • subcutaneous calcinosis
  • dyspnoea
  • dry crackles at lung bases
  • tendon friction rub
  • abrupt onset moderate/marked hypertension

Other diagnostic factors

  • fatigue
  • dry cough
  • decreased exercise tolerance
  • weight loss
  • inflammatory arthritis
  • proximal muscular weakness (inflammatory myositis)
  • synovitis
  • increased accentuation of the pulmonic component of S2 heart sound
  • signs of anaemia

Risk factors

  • FHx of scleroderma
  • immune dysregulation (e.g., positive ANA)
  • exposure to environmental substances and toxins (e.g., silica dust or solvents)

Diagnostic investigations

1st investigations to order

  • serum auto-antibodies
  • FBC
  • urea and serum creatinine
  • ESR
  • C-reactive protein (CRP)
  • urine microscopy
  • complete PFTs (spirometry, lung volumes, and diffusing capacity measurement)
  • ECG
  • echocardiogram
  • CXR
  • barium swallow
More 1st investigations to order

Investigations to consider

  • high-resolution CT scan of chest
  • upper GI endoscopy ± biopsy
  • serum muscle enzymes
  • EMG/nerve conduction studies
  • muscle biopsy
More investigations to consider

Treatment algorithm


Maureen D. Mayes

Professor of Medicine

Division of Rheumatology and Clinical Immunogenetics

University of Texas Health Science Center

Houston Medical School




MDM is an advisory board member for Boehringer-Ingelheim, Genentech and Astellas. She receives royalties from Oxford University Press for "The Scleroderma Book: A guide for patients and families". MDM is an author of a number of references cited in this monograph.

Dr Maureen D. Mayes would like to gratefully acknowledge Dr Rajpreet K. Arora-Singh, a previous contributor to this monograph. RKA declares that she has no competing interests.

Peer reviewersVIEW ALL

Associate Professor of Medicine

Department of Rheumatology

Medical College of Wisconsin




MEC has been reimbursed by: Actelion International (and is a member of its speakers bureau); Encysive Pharmaceuticals; and MediQuest Therapeutics. She is local principal investigator for the SCOT trial, sponsored by NIH.

Associate Professor

Division of Rheumatic and Autoimmune Diseases

Department of Medicine

University of Minnesota




JM declares that he has no competing interests.

Consultant Rheumatologist

Department of Rheumatology

Freeman Hospital

Newcastle Upon Tyne



BG declares that she has no competing interests.

Reader in Rheumatology and Consultant Rheumatologist

Musculoskeletal Research Group

University of Manchester




ALH declares that she has no competing interests.

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