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Systemic sclerosis (scleroderma)

Last reviewed: 25 Jun 2024
Last updated: 03 Nov 2023



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
Full details

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
Full details

Risk factors

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

Diagnostic tests

1st tests 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
  • chest x-ray
  • barium swallow
Full details

Tests to consider

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

Treatment algorithm


scleroderma renal crisis

cardiac tamponade


Raynaud phenomenon: no ulcers

Raynaud phenomenon: with digital ulcer development

generalized skin involvement

GI involvement


synovitis, tendon friction rub, or inflammatory arthritis

interstitial lung disease

pericardial effusion

pulmonary arterial hypertension



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.

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