RADIOLOGIC MANIFESTATIONS OF THE SYSTEMIC AUTOIMMUNE DISEASES
Section snippets
SYSTEMIC LUPUS ERYTHEMATOSUS
The majority of patients with systemic lupus erythematosus (SLE) develop pleural or pulmonary disease at some time during the course of their illness.21, 65 The most common radiographic manifestation is pleural effusion,65 which can be unilateral or bilateral and is frequently associated with pericardial effusion (Fig. 1).58 Although most pleural effusions resolve completely, some residual pleural thickening may persist.
Although pleural disease is more frequently seen, pulmonary parenchymal
RHEUMATOID ARTHRITIS
Pleural involvement is probably the most common thoracic manifestation of rheumatoid arthritis. Although pleural effusions occur, pleural thickening is a more frequently seen radiographic finding.62 Pleural disease is much more common in male patients and is usually asymptomatic.26 Pleural effusions are usually unilateral and are often loculated (Fig. 4). Although pleural effusions usually occur late in the disease, they do not correlate with severity of arthritis and may occasionally develop
SJÖGREN'S SYNDROME
Interstitial fibrosis occurs in Sjögren's syndrome and has a similar pattern and distribution as other collagen vascular diseases.29, 54 Parenchymal abnormalities consistent with interstitial fibrosis are seen on chest radiograph in 10% to 14% of cases.18, 31, 54 Patients with Sjögren's syndrome also have an increased incidence of lymphocytic interstitial pneumonitis that presents radiographically as a reticulonodular pattern involving predominantly the lower lobes.56 Patients with Sjögren's
POLYMYOSITIS/DERMATOMYOSITIS
The most common radiographic abnormality is probably aspiration pneumonia secondary to pharyngeal muscle weakness. It occurs in approximately 50% of patients.11, 49 Radiographic findings of aspiration pneumonia consist of segmental areas of air space consolidation that may be unilateral or bilateral and involve the dependent lung regions. Involvement of the diaphragm leads to diaphragmatic elevation, small lung volumes, and basilar linear atelectasis.47
Interstitial fibrosis with a pattern and
PROGRESSIVE SYSTEMIC SCLEROSIS
Pulmonary disease is common in patients with progressive systemic sclerosis (PSS, scleroderma). Interstitial fibrosis is the most common radiographic abnormality, being present in 20% to 65% of patients.3, 48 The fibrosis usually has a basilar predominance, initially presenting as a fine reticular pattern that progresses to coarse reticulation and honeycombing (Fig. 10).3 High resolution CT scanning may demonstrate evidence of fibrosis in patients with normal radiographs.23, 48 In a prospective
ANKYLOSING SPONDYLITIS
Apical fibrosis has been described as the most common pulmonary abnormality evident on the chest radiograph in patients with ankylosing spondylitis. In a study of chest radiographic findings in 2080 patients with ankylosing spondylitis, 26 (1.2%) had fibrosis in the upper lobes.45 The radiographic findings consist of reticulonodular opacities in the lung apices, which become confluent as the disease progresses.7, 45, 66 Common associated abnormalities include apical bullae and cavities (Fig. 12)
WEGENER'S GRANULOMATOSIS
Wegener's granulomatosis (WG) is a systemic autoimmune disorder characterized by necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts, necrotizing glomerulonenephritis, and small vessel vasculitis. During the course of the disease, most patients have lung involvement. In a study of 158 patients with WG, the chest radiograph was abnormal at presentation in 45% of patients and eventually abnormal in 85% of patients.24 The most common radiographic findings are
CHURG-STRAUSS SYNDROME
Churg-Strauss syndrome is a systemic necrotizing vasculitis that occurs almost exclusively in patients with asthma. The chest radiograph is abnormal in the majority of patients. In one review of 138 cases, pulmonary opacities were present on chest radiographs in 74% of patients.32 The radiographic appearances are variable. The most common findings are patchy nonsegmental areas of consolidation with no zonal predominance. The abnormalities are often transient and may have a peripheral
ANTIGLOMERULAR BASEMENT MEMBRANE DISEASE
Antiglomerular basement membrane disease is defined by a triad of diffuse pulmonary hemorrhage, glomerulonephritis, and circulating antiglomerular basement membrane antibodies. The chest radiograph typically demonstrates extensive bilateral air-space consolidation5 (Fig. 18). The consolidation usually is symmetric and has a perihilar predominance with a tendency to spare the lung apices. The consolidation usually resolves within 2 to 3 days and is replaced by a reticulonodular pattern and
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Cited by (22)
Connective Tissue Disease-related Thoracic Disease
2015, Clinics in Chest MedicineCitation Excerpt :The most common cause of a pulmonary opacity in SLE is infection. Pulmonary hemorrhage and pulmonary thromboembolism may also occur (Box 5).59,60 Pleuritis is the most specific thoracic manifestation, occurring in 40% to 85% of patients with SLE,61–63 and is one of the diagnostic criteria for SLE (Fig. 8).64
Clinical Aspects of Ankylosing Spondylitis
2006, Ankylosing Spondylitis and the Spondyloarthropathies: A Companion to Rheumatology 3EClinical Aspects of Ankylosing Spondylitis
2006, Ankylosing Spondylitis and the SpondyloarthropathiesHigh Resolution Computed Tomography of the Lungs
2004, Handbook of Systemic Autoimmune DiseasesCitation Excerpt :Other infections occasionally described in patients with SLE include tuberculosis and other mycobacterial infections, Pneumocystis carinii, Rhodococcus spp., and Pseudomonas spp. Acute lupus pneumonitis, hemorrhage, ARDS, and OP can result in similar radiologic findings (Onomura et al., 1991; Wiederman and Matthay, 1992; Bankier et al., 1995; Primack and Müller, 1998; Franquet, 2001). In patients with ankylosing spondylitis, cavities may develop within distorted fibrotic apical tissue.
Drug-Induced Respiratory Disease in Connective Tissue Diseases
2004, Handbook of Systemic Autoimmune DiseasesCitation Excerpt :(6) Exclusion of other causes for the lung disease. This is a difficult exercise, especially in patients with infiltrative lung disease (ILD), because drugs and the background disease can produce similar imaging-pathologic patterns of parenchymal involvement (Prakash, 1984; Murin et al., 1998; Primack and Muller, 1998; Schwarz, 1998; Sullivan and Hoffman, 1998; Tanoue, 1998; Mayberry et al., 2000; Franquet, 2001; Freemer and King, 2003). Adequate sampling of blood and lung fluids should be performed to exclude an infection (Shelhamer et al., 1996).
Approach to ground-glass opacification of the lung
2002, Seminars in Ultrasound CT and MRI
Address reprint requests to Nestor L. Müller, MD, PhD, Department of Radiology, Vancouver Hospital and Health Sciences Centre, University of British Columbia, 855 W. 12th Avenue, Vancouver, British Columbia, Canada V5Z 1M9