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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Systemic sclerosis
Definitions
- Generalized connective tissue disorder affecting multiple organs, including skin, lungs, heart, and kidneys
- Limited cutaneous systemic sclerosis (60%)
- Skin involvement: Hands, forearms, feet, face
- Longstanding Raynaud phenomenon
- CREST syndrome: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias
- Diffuse cutaneous systemic sclerosis (40%)
- Acute onset: Raynaud phenomenon, 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
- Chung JH et al: Imaging features of systemic sclerosis-associated interstitial lung disease. J Vis Exp. 2020
- Perelas A et al: Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 8(3):304-20, 2020
- Sanchez F et al: Pathological entities that may affect the lungs and the myocardium. Evaluation with chest CT and cardiac MR. Clin Imaging. 70:124-35, 2020
- Elicker BM et al: Imaging of the thoracic manifestations of connective tissue disease. Clin Chest Med. 40(3):655-66, 2019
- Kusmirek JE et al: Thoracic manifestations of connective tissue diseases. Semin Ultrasound CT MR. 40(3):239-54, 2019
- Strollo D et al: Imaging lung disease in systemic sclerosis. Curr Rheumatol Rep. 12(2):156-61, 2010
- Lynch DA: Lung disease related to collagen vascular disease. J Thorac Imaging. 24(4):299-309, 2009
- de Azevedo AB et al: Prevalence of pulmonary hypertension in systemic sclerosis. Clin Exp Rheumatol. 23(4):447-54, 2005
- Galie N et al: Pulmonary arterial hypertension associated to connective tissue diseases. Lupus. 14(9):713-7, 2005
- Highland KB et al: New Developments in Scleroderma Interstitial Lung Disease. Curr Opin Rheumatol. 17(6):737-45, 2005
- 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
- Kim EA et al: Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics. 2002 Oct;22 Spec No:S151-65. Review. Erratum in: Radiographics. 23(5):1340, 2003
- Kim EA et al: Interstitial pneumonia in progressive systemic sclerosis: serial high-resolution CT findings with functional correlation. J Comput Assist Tomogr. 25(5):757-63, 2001
- Diot E et al: Relationship between abnormalities on high-resolution CT and pulmonary function in systemic sclerosis. Chest. 114(6):1623-9, 1998
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Systemic sclerosis
Definitions
- Generalized connective tissue disorder affecting multiple organs, including skin, lungs, heart, and kidneys
- Limited cutaneous systemic sclerosis (60%)
- Skin involvement: Hands, forearms, feet, face
- Longstanding Raynaud phenomenon
- CREST syndrome: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias
- Diffuse cutaneous systemic sclerosis (40%)
- Acute onset: Raynaud phenomenon, 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
- Chung JH et al: Imaging features of systemic sclerosis-associated interstitial lung disease. J Vis Exp. 2020
- Perelas A et al: Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 8(3):304-20, 2020
- Sanchez F et al: Pathological entities that may affect the lungs and the myocardium. Evaluation with chest CT and cardiac MR. Clin Imaging. 70:124-35, 2020
- Elicker BM et al: Imaging of the thoracic manifestations of connective tissue disease. Clin Chest Med. 40(3):655-66, 2019
- Kusmirek JE et al: Thoracic manifestations of connective tissue diseases. Semin Ultrasound CT MR. 40(3):239-54, 2019
- Strollo D et al: Imaging lung disease in systemic sclerosis. Curr Rheumatol Rep. 12(2):156-61, 2010
- Lynch DA: Lung disease related to collagen vascular disease. J Thorac Imaging. 24(4):299-309, 2009
- de Azevedo AB et al: Prevalence of pulmonary hypertension in systemic sclerosis. Clin Exp Rheumatol. 23(4):447-54, 2005
- Galie N et al: Pulmonary arterial hypertension associated to connective tissue diseases. Lupus. 14(9):713-7, 2005
- Highland KB et al: New Developments in Scleroderma Interstitial Lung Disease. Curr Opin Rheumatol. 17(6):737-45, 2005
- 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
- Kim EA et al: Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiographics. 2002 Oct;22 Spec No:S151-65. Review. Erratum in: Radiographics. 23(5):1340, 2003
- Kim EA et al: Interstitial pneumonia in progressive systemic sclerosis: serial high-resolution CT findings with functional correlation. J Comput Assist Tomogr. 25(5):757-63, 2001
- Diot E et al: Relationship between abnormalities on high-resolution CT and pulmonary function in systemic sclerosis. Chest. 114(6):1623-9, 1998
