Related Experiment Videos
Progressive systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis
R M Lomeo1, R J Cornella, S I Schabel
1Department of Medicine, Medical University of South Carolina, Charleston 29425.
The American Journal of Medicine
|November 1, 1989
Summary
Systemic sclerosis sine scleroderma, a form of scleroderma without skin involvement, can cause lung disease. Early diagnosis is possible with specific tests even without skin symptoms.
Area of Science:
- Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Systemic sclerosis sine scleroderma (SSS) is a rare subset of systemic sclerosis (SSc).
- SSS presents with internal organ involvement, notably lung disease, without characteristic skin thickening.
- Diagnosing SSS can be challenging due to the absence of cutaneous manifestations.
Purpose of the Study:
- To describe the clinical characteristics of 10 patients with SSS and lung disease.
- To highlight diagnostic challenges and facilitate earlier recognition of SSS.
Main Methods:
- Retrospective review of patients with SSS and interstitial lung disease (ILD).
- Inclusion criteria: clinical/radiographic ILD without skin involvement.
- MEDLINE search for patients with ILD who later developed scleroderma.
Main Results:
- Ten male patients with SSS and pulmonary symptoms were identified.
- Common findings included positive antinuclear antibody, esophageal dysmotility, restrictive lung disease, Raynaud's phenomenon, and abnormal nailfold capillaries.
- Six patients developed overt scleroderma within 7 years; lung biopsies showed interstitial pneumonitis and fibrosis.
Conclusions:
- Systemic sclerosis should be considered in idiopathic interstitial lung disease, even without skin signs.
- Diagnostic tools like esophagram, antinuclear antibody testing, and nailfold capillaroscopy are crucial for early SSS diagnosis.
- This presentation of SSc predominantly affects men.