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
Related Anatomy
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Related Differential Diagnoses
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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
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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