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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Progressive systemic sclerosis (PSS)
Synonyms
- Scleroderma
- Systemic sclerosis
Definitions
- Generalized autoimmune connective tissue disorder affecting multiple organ systems including skin, lungs, heart, and kidneys
- Limited cutaneous systemic sclerosis (60%)
- Skin involvement of hands, forearms, feet, and face
- Longstanding Raynaud phenomenon
- CREST syndrome: Calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, and telangiectasias
- Diffuse cutaneous systemic sclerosis (40%)
- Acute onset: Raynaud, acral and truncal skin involvement
- High frequency of interstitial lung disease
- Scleroderma sine scleroderma (rare)
- Interstitial lung disease without skin manifestations
IMAGING
General Features
Radiographic Findings
CT Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Capobianco J et al: Thoracic manifestations of collagen vascular diseases. Radiographics. 32(1):33-50, 2012
- Goldin JG et al: High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 134(2):358-67, 2008
- Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
- Wells AU: High-resolution computed tomography and scleroderma lung disease. Rheumatology (Oxford). 47 Suppl 5:v59-61, 2008
- Desai SR et al: CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology. 232(2):560-7, 2004
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Progressive systemic sclerosis (PSS)
Synonyms
- Scleroderma
- Systemic sclerosis
Definitions
- Generalized autoimmune connective tissue disorder affecting multiple organ systems including skin, lungs, heart, and kidneys
- Limited cutaneous systemic sclerosis (60%)
- Skin involvement of hands, forearms, feet, and face
- Longstanding Raynaud phenomenon
- CREST syndrome: Calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, and telangiectasias
- Diffuse cutaneous systemic sclerosis (40%)
- Acute onset: Raynaud, acral and truncal skin involvement
- High frequency of interstitial lung disease
- Scleroderma sine scleroderma (rare)
- Interstitial lung disease without skin manifestations
IMAGING
General Features
Radiographic Findings
CT Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Capobianco J et al: Thoracic manifestations of collagen vascular diseases. Radiographics. 32(1):33-50, 2012
- Goldin JG et al: High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 134(2):358-67, 2008
- Madani G et al: The role of radiology in the management of systemic sclerosis. Clin Radiol. 63(9):959-67, 2008
- Wells AU: High-resolution computed tomography and scleroderma lung disease. Rheumatology (Oxford). 47 Suppl 5:v59-61, 2008
- Desai SR et al: CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology. 232(2):560-7, 2004
