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
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
Synonyms
Systemic sclerosis
Definitions
Generalized connective tissue disorder affecting multiple organs, including skin, lungs, heart, and kidneys
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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