Last reviewed: 19 Dec 2021
Last updated: 25 Aug 2020



History and exam

Key diagnostic factors

  • Raynaud phenomenon
  • digital pits or ulcers
  • swelling of the hands and feet
  • skin thickening
  • loss of function of hands
  • sclerodactyly
  • heartburn, reflux, and dysphagia
  • bloating
  • fecal incontinence
  • arthralgias and myalgias
  • abnormal nail-fold capillaroscopy
  • telangiectasia
  • subcutaneous calcinosis
  • dyspnea
  • 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 anemia

Risk factors

  • famliy history 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 autoantibodies
  • CBC
  • BUN and serum creatinine
  • ESR
  • CRP
  • urine microscopy
  • complete pulmonary function tests (spirometry, lung volumes, and diffusing capacity measurement)
  • ECG
  • echocardiogram
  • CXR
  • barium swallow

Investigations to consider

  • high-resolution CT scan of chest
  • upper gastrointestinal endoscopy ± biopsy
  • serum muscle enzymes
  • electromyogram/nerve conduction studies
  • muscle biopsy

Treatment algorithm



Maureen D. Mayes, MD, MPH
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, Galapagos, Corbus, and Eicos. MDM is on the grant review board for Medtelligence. She receives royalties from Oxford University Press and Henry Stewart Talks. MDM is an author of a number of references cited in this topic.


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

Peer reviewers

Mary Ellen Csuka, MD

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.

Jerry Molitor, MD, PhD

Associate Professor

Division of Rheumatic and Autoimmune Diseases

Department of Medicine

University of Minnesota




JM declares that he has no competing interests.

Bridget Griffiths, MD, FRCP(UK)

Consultant Rheumatologist

Department of Rheumatology

Freeman Hospital

Newcastle Upon Tyne



BG declares that she has no competing interests.

Ariane L. Herrick, MD, FRCP

Reader in Rheumatology and Consultant Rheumatologist

Musculoskeletal Research Group

University of Manchester




ALH declares that she has no competing interests.

Use of this content is subject to our disclaimer