Collagen Diseases and Vasculitis

6 Collagen Diseases and Vasculitis


Rheumatoid Arthritis


Definition


Generalized inflammatory joint disorder of uncertain etiology.


image Epidemiology


Most common collagen vascular disease image Extraarticular manifestations are rare image More common in men than women image Rarely occurs before joint symptoms image Occurs in middle age.


image Etiology, pathophysiology, pathogenesis


Etiology is unclear image Pleuropulmonary manifestations include pleural involvement and, less pronounced, fibrosing alveolitis, rheumatoid nodules, airway changes (bronchitis, bronchiolitis, bronchiectasis).


Imaging Signs


image Modality of choice


CT.


image Radiographic findings


Low sensitivity image Severe cases show predominantly basal reticulonodular shadowing and linear, nonseptal opacities image Honeycombing image Pleuritis (pleural effusion image Rheumatoid nodules (< 5% of cases).


image CT findings


Pathologic findings are visualized in about 30% of cases.


Pleural findings (most common): Include slight pleural effusion image Pleural thickening.


Parenchymal findings (predominantly basal and subpleural): Include finely to coarsely reticular and nodular opacities with honeycombing, similar to idiopathic pulmonary fibrosis or nonspecific interstitial pneumonitis image Ground-glass opacities image Rheumatoid nodules (solitary or multiple, peripheral, sharply demarcated, 50% showing cavitation, rarely calcified).


Airway findings: Include bronchiolitis image Bronchiectasis.


image Pathognomonic findings


Coarsely reticular and nodular interstitial changes in combination with joint symptoms.


Clinical Aspects


image Typical presentation


Presentation is variable; patients may be asymptomatic image Dyspnea with nonproductive image Pleuritis image Lung function is restricted with impaired diffusion capacity and reduced vital capacity image Positive rheumatoid factor in 80% of cases image Pleural exudate is high in protein and low in glucose with a high level of lactate dehydrogenase and low pH and contains lymphocytic, neutrophilic, and eosinophilic cells.


image Therapeutic options


Anti-inflammatory treatment of the underlying disorder (anti-inflammatory agents, steroids, TNF-α blockers, immunosuppressives).


image


Fig. 6.1 Plain chest radiograph of a 70-year-old man with a long history of rheumatoid arthritis. Relatively nonspecific bilateral basal finely reticular streaky shadowing consistent with pulmonary interstitial changes.


image Course and prognosis


Depend on the underlying disorder.


image What does the clinician want to know?


Characterize and ascertain the extent of findings.


Differential Diagnosis
















Idiopathic pulmonary fibrosis


– Ambiguous radiographic morphology


– No pleural changes


– No rheumatoid nodules


– No joint pathology


Scleroderma


– Ambiguous radiographic morphology


– Esophageal dilation


– No joint pathology


Asbestosis


– Ambiguous radiographic morphology


– Pleural plaques


– History of occupational exposure


Tips and Pitfalls


In the presence of a known underlying disorder, the diagnosis is straightforward image However, the findings themselves are nonspecific image Focal lesions require histologic evaluation.


image


Fig. 6.2 Rheumatoid nodules in a 70-year-old woman.


a The plain chest radiograph shows bilateral focal lesions, the largest of which occur in the left laterobasal pleural region.


b Findings on CT include isolated eccentric cavities and bronchiectatic changes.


Selected References

Biederer J et al. Correlation between HRCT findings, pulmonary function tests and bronchoalveolar lavage cytology in interstitial lung disease associated with rheumatoid arthritis. Eur Radiol 2004; 14: 272–280


Remy-Jardin M et al. Lung changes in rheumatoid arthritis: CT findings. Radiology 1994; 193: 375–382


Systemic Lupus Erythematosus


Definition


image Epidemiology


Systemic autoimmune disorder, a member of the group of collagen diseases.


image Etiology, pathophysiology, pathogenesis


Antinuclear antibodies are present in 95% of cases image Genetic disposition image 90% of patients are women image Pleuropulmonary involvement in about 50% of cases, usually in the form of pleural effusions, less often as acute lupus pneumonitis with damage to the alveolar-capillary membrane and alveolar hemorrhages image Pulmonary fibrosis rarely develops.


Imaging Signs


image Modality of choice


CT is preferable to plain radiography.


image Radiographic and CT findings


Pleuritis or pleural effusion in 70% of cases, pericardial effusion in 35% (usually slight and bilateral) image Lupus pneumonitis in 5% with nodular consolidations, ground-glass opacities, alveolar hemorrhages, predominantly basal image “Shrinking lung” syndrome: increasing volume loss without significant parenchymal changes (from diaphragmatic dysfunction or pleuritis) image Fibrosis, thickening of the bronchial wall, or bronchiectasis are rare image Note: Pneumonia is common in immunosuppression (whether pathologic or drug-induced).


image Pathognomonic findings


There are no pathognomonic findings. Pleural and pulmonary changes tend to be nonspecific.


Clinical Aspects


image Typical presentation


Presence of at least four of the following symptoms is diagnostic: butterfly erythema, discoid lupus, photosensitivity, oral ulcerations, arthritis, serositis, renal involvement, CNS involvement, antinuclear antibodies image With pulmonary involvement, symptoms include fever, dyspnea, cough, chest pain, and hemoptysis.


image Therapeutic options


Immunosuppressives.


image Course and prognosis


Depend on the organs involved.


image


Fig. 6.3


a     Pleuritis with systemic lupus erythematosus. The plain chest radiograph of a patient with reduced depth of inspiration shows a left pleural effusion with strips of dystelectasis on the right.


b, c The CT scans show pericardial involvement in addition to the pleural effusion but no interstitial or parenchymal changes.


image


Fig. 6.4 CT in systemic lupus erythematosus showing discrete inter-stitial changes resembling ground-glass opacity.


Differential Diagnosis
















Other collagen diseases


– Clinical findings are crucial to the diagnosis


– Morphologic findings on the radiograph are usually ambiguous


Idiopathic interstitial pneumonia


– Pulmonary changes are most important and are crucial to the diagnosis


Pneumonia


– In systemic lupus erythematosus there is usually other pulmonary pathology with pleural involvement, pulmonary hemorrhage, and interstitial changes


Tips and Pitfalls

Stay updated, free articles. Join our Telegram channel

Feb 28, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on Collagen Diseases and Vasculitis

Full access? Get Clinical Tree

Get Clinical Tree app for offline access