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Imaging Manifestations of Collagen Vascular Disease Steven Primack, MD
THORACIC MANIFESTATONS of COLLAGEN VASCULAR DISEASE Steven L. Primack, MD Department of Radiology Oregon Health and Science University
Objectives • Review spectrum of thoracic involvement in patients with collagen vascular disease • Differences in thoracic manifestations of the various collagen vascular diseases
Collagen Vascular Disease • • • • • •
Systemic lupus erythematosus (SLE) Rheumatoid arthritis Progressive systemic sclerosis (scleroderma) Polymyositis / Dermatomyositis Sjögren’s syndrome Mixed connective tissue disease
• • • • • •
SLE • 20-50 y/o women:men, 10:1 • Thoracic involvement in >50% • Primary and secondary manifestations • Pleural dz by far most common • Infection relatively common • ILD uncommon, < 5%
SLE
SLE
Primary Thoracic Manifestations
Secondary Thoracic Manifestations
Pleuritis, pericarditis Acute lupus pneumonitis Pulmonary hemorrhage ILD: NSIP>>UIP, UIP • PA HTN • Diaphragmatic dysfunction • Aspiration
Collagen Vascular Disease • NSIP/UIP: • • • • •
PSS, PM/DM, RA SLE, Sjögren’s Pleural: SLE, RA OP: PM/DM, RA BO: RA Bronchiectasis: RA, Sjögren’s Follicular bronchiolitis: RA, Sjögren’s
Collagen Vascular Disease • Aspiration: • Diaphragm:
PM/DM, scleroderma PM/DM, SLE
• • • •
pneumonia, OP increased with fibrosis Sjögren’s PSS and MCTD >> SLE
Consolidation: Lung Cancer: LIP, lymphoma: PA HTN:
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Suggested Reading • Jokerst C, et al: An overview of collagen vascular disease – associated interstitial disease. Seminars in Roentgenology 50(1): 31-39, 2015. • Capobianco J, et al: Thoracic manifestations of collagen vascular diseases. RadioGraphics 32: 33-50, 2012. • Lynch DA: Lung disease related to collagen vascular disease. J Thoracic Imaging 24(4): 299-309, 2009. • Kim EA, et al: Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. RadioGraphics 22: S151S165, 2002. • Mayberry JP, et al: Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. RadioGraphics 20: 1623-1635, 2000.