6 Collagen Diseases and Vasculitis Generalized inflammatory joint disorder of uncertain etiology. Epidemiology Most common collagen vascular disease Extraarticular manifestations are rare More common in men than women Rarely occurs before joint symptoms Occurs in middle age. Etiology, pathophysiology, pathogenesis Etiology is unclear Pleuropulmonary manifestations include pleural involvement and, less pronounced, fibrosing alveolitis, rheumatoid nodules, airway changes (bronchitis, bronchiolitis, bronchiectasis). Modality of choice CT. Radiographic findings Low sensitivity Severe cases show predominantly basal reticulonodular shadowing and linear, nonseptal opacities Honeycombing Pleuritis (pleural effusion Rheumatoid nodules (< 5% of cases). CT findings Pathologic findings are visualized in about 30% of cases. – Pleural findings (most common): Include slight pleural effusion 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 Ground-glass opacities Rheumatoid nodules (solitary or multiple, peripheral, sharply demarcated, 50% showing cavitation, rarely calcified). – Airway findings: Include bronchiolitis Bronchiectasis. Pathognomonic findings Coarsely reticular and nodular interstitial changes in combination with joint symptoms. Typical presentation Presentation is variable; patients may be asymptomatic Dyspnea with nonproductive Pleuritis Lung function is restricted with impaired diffusion capacity and reduced vital capacity Positive rheumatoid factor in 80% of cases 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. Therapeutic options Anti-inflammatory treatment of the underlying disorder (anti-inflammatory agents, steroids, TNF-α blockers, immunosuppressives). Course and prognosis Depend on the underlying disorder. What does the clinician want to know? Characterize and ascertain the extent of findings.
Rheumatoid Arthritis
Definition
Imaging Signs
Clinical Aspects
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 However, the findings themselves are nonspecific Focal lesions require histologic evaluation.
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
Epidemiology
Systemic autoimmune disorder, a member of the group of collagen diseases.
Etiology, pathophysiology, pathogenesis
Antinuclear antibodies are present in 95% of cases Genetic disposition 90% of patients are women 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 Pulmonary fibrosis rarely develops.
Imaging Signs
Modality of choice
CT is preferable to plain radiography.
Radiographic and CT findings
Pleuritis or pleural effusion in 70% of cases, pericardial effusion in 35% (usually slight and bilateral) Lupus pneumonitis in 5% with nodular consolidations, ground-glass opacities, alveolar hemorrhages, predominantly basal “Shrinking lung” syndrome: increasing volume loss without significant parenchymal changes (from diaphragmatic dysfunction or pleuritis) Fibrosis, thickening of the bronchial wall, or bronchiectasis are rare Note: Pneumonia is common in immunosuppression (whether pathologic or drug-induced).
Pathognomonic findings
There are no pathognomonic findings. Pleural and pulmonary changes tend to be nonspecific.
Clinical Aspects
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 With pulmonary involvement, symptoms include fever, dyspnea, cough, chest pain, and hemoptysis.
Therapeutic options
Immunosuppressives.
Course and prognosis
Depend on the organs involved.
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 |