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Clinical aspects of systemic and localized scleroderma
F H van den Hoogen1, E M de Jong
1Department of Rheumatology, University Hospital Nijmegen, The Netherlands.
Current Opinion in Rheumatology
|November 1, 1995
Summary
Systemic sclerosis frequently affects the gastrointestinal tract, with symptoms appearing early. Internal organ involvement is rare in localized scleroderma, but extremity cases can cause long-term disability.
Area of Science:
- Rheumatology
- Gastroenterology
- Dermatology
Background:
- The gastrointestinal tract is the second most commonly affected organ in systemic sclerosis (SSc).
- Gastrointestinal symptoms in SSc can manifest early and may not correlate with objective findings.
- Esophageal dysmotility, while common in SSc, is not specific and also occurs in other connective tissue diseases.
Purpose of the Study:
- To review the various organ involvements in systemic sclerosis.
- To highlight the gastrointestinal tract as a primary site of SSc manifestation.
- To discuss the clinical presentation and associations of SSc organ involvement.
Main Methods:
- Literature review of systemic sclerosis and its organ manifestations.
- Analysis of clinical findings in patients with limited cutaneous SSc and CREST variant.
- Examination of pulmonary and peripheral nerve involvement in SSc cohorts.
Main Results:
- Gastrointestinal symptoms are common in SSc, with esophageal dysmotility being non-specific.
- Peripheral macrovascular disease and autonomic dysfunction are noted in specific SSc subtypes (lcSSc, CREST).
- Pulmonary involvement affects 40% of patients; peripheral nerve involvement is often subclinical and associated with specific antibodies.
Conclusions:
- Systemic sclerosis involves multiple organs, with the GI tract being frequently affected.
- Organ involvement varies by SSc subtype and can include vascular, autonomic, pulmonary, and nervous systems.
- Localized scleroderma, especially in childhood extremities, can lead to significant functional disability.