Initial symptoms are non-specific and include fatigue, vague musculoskeletal complaints, diffuse swelling of hands, and Raynaud's phenomenon.

Aetiology and pathogenesis are unknown.

Disease course is variable, but the condition rarely subsides spontaneously.

There are two main subtypes of systemic sclerosis (SSc): limited cutaneous SSc and diffuse cutaneous SSc. The limited cutaneous form tends to have less severe internal organ involvement and a better prognosis, but these subjects still need to be followed closely for possible complications.

Clinical course is determined by extent of vascular and fibrosing complications. Vascular involvement includes Raynaud's phenomenon, ischaemic digital ulcers, hypertensive crisis, and pulmonary arterial hypertension. Fibrosis can involve lungs, heart, and GI tract.

Treatment is targeted on disease processes that are potentially reversible (e.g., active inflammation or vasoconstriction) and aims to minimise functional impairment of the patient.

Systemic sclerosis (SSc), also known as scleroderma, is a multi-system, autoimmune disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of auto-antibodies.

History and exam

Key diagnostic factors

Full details

Other diagnostic factors

Risk factors

Diagnostic investigations

1st investigations to order

Investigations to consider

Treatment algorithm

scleroderma renal crisis

cardiac tamponade

Raynaud's phenomenon: no ulcers

Raynaud's phenomenon: with digital ulcer development

generalised skin involvement

GI involvement

synovitis, tendon friction rub, or inflammatory arthritis

interstitial lung disease

pericardial effusion

pulmonary arterial hypertension

Authors VIEW ALL 

Maureen D. Mayes, MD, MPH 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 reviewers VIEW ALL 

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)
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