Imaging Manifestations of Collagen Vascular Disease

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1 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 () Rheumatoid arthritis Progressive systemic sclerosis (scleroderma) Polymyositis / Dermatomyositis Sjögren s syndrome Mixed connective tissue disease 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% Primary Thoracic Manifestations Pleuritis, pericarditis Acute lupus pneumonitis Pulmonary hemorrhage ILD: NSIP>>UIP, <5%, usually mild Diaphragm dysfunction PA HTN Secondary Thoracic Manifestations Pneumonia: community acquired, opportunistic Atelectasis Drug-induced lung disease Renal failure, pulmonary edema

2 Pleural Disease - Pleural Effusion SUNDAY Up to 50% during course of illness Unilateral or bilateral effusions Most effusions resolve, some residual thickening Associated pericardial effusion Ddx: parapneumonic, nephrotic syndrome, PE Pleural and Pericardial Effusion Acute Lupus Pneumonitis Incidence 1 4%, usually established disease DAD, necrosis, alveolar hemorrhage Mimics pneumonia, infarction, hemorrhage Patchy consolidation, usually bilateral Diagnosis of exclusion

3 Pulmonary Hemorrhage Pulmonary Hemorrhage Incidence <3%, usually established disease Rapid onset dyspnea, fever, hemoptysis Diffuse bilateral ground glass or consolidation Mortality 40-90% Pulmonary Hemorrhage Pneumocystis Pneumonia Diaphragm Dysfunction Diaphragmatic weaknesss due to myopathy Incidence: ~20% Usually bilateral hemidiaphragm elevation Shrinking lung syndrome

4 Diaphragm Dysfunction Rheumatoid Arthritis SUNDAY y/o, women:men, 3:1 Thoracic disease more common in men Extraarticular involvement in 50% 4 years later Rheumatoid Arthritis Thoracic Manifestations Pleural disease Bronchiectasis, BO (consider expiratory scan) UIP slightly more common than NSIP Organizing pneumonia Follicular bronchiolitis Necrobiotic nodules Rheumatoid Arthritis Pleural Disease Pleural effusion usually unilateral, often loculated Pleural thickening very common, up to 50% at autopsy 4% before, 20% with onset of joint disease Associated pericarditis, subcutaneous nodules

5 RA Pleural Thickening Rheumatoid Arthritis RA - UIP Nonspecific Interstitial Pneumonitis NSIP Fibrotic or cellular NSIP HRCT: ground-glass opacity, consolidation, reticulation, traction bronchiectasis Peripheral and lower lung zone May have subpleural sparing Honeycombing generally absent RA - NSIP RA - NSIP

6 RA interval increase in GGO RA NSIP, Pneumocystis Pneumonia SUNDAY RA Necrobiotic Nodules Rare, <0.5% incidence on CXR Advanced RA and subcutaneous nodules Wax and wane with arthritis Cavitation common, irregular margins Rheumatoid Arthritis: Organizing Pneumonia RA Follicular Bronchiolitis Progressive Systemic Sclerosis (Scleroderma) Inflammatory, fibrotic changes of lungs, skin, vasculature, visceral organs Limited and diffuse forms y/o women:men, 3:1 8:1

7 Scleroderma Thoracic Manifestations Pulmonary fibrosis: 30-90% patients - NSIP >> UIP Esophageal dysmotility - dilated, air-filled esophagus, aspiration Follicular bronchiolitis PA HTN: ILD, direct vascular involvement Highest mortality of CVDs: PA HTN Scleroderma - NSIP 33F 33F with scleroderma 33F Supine Scleroderma UIP Prone NSIP

8 Polymyositis / Dermatomyositis SUNDAY Inflammatory myopathies of skeletal muscle Dermatomyositis additional skin changes y/o women:men, 2:1 Progressive weakness of proximal muscles Hypoventilation, respiratory failure Polymyositis/Dermatomyositis Thoracic Manifestations Polymyositis NSIP Diaphragm and intercostal muscle weakness elevated diaphragm, basilar atelectasis Pharyngeal muscle weakness aspiration ILD: NSIP and OP most common, often coexist - UIP also occurs - ILD in 30-60%, most frequent if anti-jo-1 Dermatomyositis 60m Dermatomyositis NSIP Atelectasis v. ILD

9 Dermatomyositis NSIP, OP Sjögren s Syndrome Lymphocyte infiltration salivary, lacrimal glands, extraglandular in 5 10% y/o women:men, 9:1 Primary or secondary (usually with RA) Lymphoma as high as 44-fold increase Sjögren s Syndrome Thoracic manifestations Airway disease most common bronchiectasis ILD - uncommon Lymphocytic interstitial pneumonitis (LIP) Follicular bronchiolitis Lymphoma enlarged nodes, multifocal masses, consolidation, pleural effusion Sjögren s Syndrome - Bronchiectasis Sjögren s Syndrome - LIP 34 F

10 LIP LIP SUNDAY Mixed Connective Tissue Disease Mixed Connective Tissue Disease - NSIP, scleroderma, RA, PM/DM Pleural effusion ILD: NSIP > UIP PA HTN Diaphragmatic dysfunction Aspiration Collagen Vascular Disease NSIP/UIP: PSS, PM/DM, RA, Sjögren s Pleural:, RA OP: PM/DM, RA BO: RA Bronchiectasis: RA, Sjögren s Follicular bronchiolitis: RA, Sjögren s Aspiration: Diaphragm: Collagen Vascular Disease PM/DM, scleroderma PM/DM, Consolidation: pneumonia, OP Lung Cancer: increased with fibrosis LIP, lymphoma: Sjögren s PA HTN: PSS and MCTD >>

11 Suggested Reading Jokerst C, et al: An overview of collagen vascular disease associated interstitial disease. Seminars in Roentgenology 50(1): 31-39, Capobianco J, et al: Thoracic manifestations of collagen vascular diseases. RadioGraphics 32: 33-50, Lynch DA: Lung disease related to collagen vascular disease. J Thoracic Imaging 24(4): , Kim EA, et al: Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. RadioGraphics 22: S151- S165, Mayberry JP, et al: Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. RadioGraphics 20: , 2000.

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