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Chest Manifestations of Systemic Diseases 104_111_Kazerooni 28-02-2007 07:48 Pagina 104 IDKD 2007 Chest Manifestations of Systemic Diseases E.A. Kazerooni Department of Radiology, Medical Center Drive, University of Michigan Medical Center, Arbor, MI, USA There are numerous systemic diseases, both acquired and and micronodules, ground-glass opacity, and honey- hereditary, that affect the thorax. The many radiologic combing. The findings may be very similar to those of manifestations of these diseases may involve the lungs the usual interstitial pneumonia (UIP) form of idiopathic and pleura as well as cardiovascular, neurologic, hemato- interstitial pneumonia; however, in scleroderma the dis- logic and osseous structures of the thorax. What follows ease progresses more slowly. Esophageal dysmotility re- is a summary of the more common of these entities. sults in a dilated air-filled esophagus and findings of as- piration pneumonia. Pulmonary arterial hypertension oc- curs in 6-60% of patients, and sclerosis of cardiac mus- Collagen Vascular Diseases cle may result in cor pulmonale [5]. Pleural involvement is rare. There is an increased risk of lung cancer (adeno- Scleroderma (Progressive Systemic Sclerosis) and bronchoalveolar). Demographics and Clinical Manifestations Rheumatoid Arthritis The incidence of scleroderma is 2-12 per million, with Demographics and Clinical Manifestations the usual onset before age 45 and a three-fold higher in- cidence in women than in men. PSS is characterized by The prevalence of RA is as high as 2%. The usual age of sclerosis and atrophy of the skin, GI tract, musculoskele- onset is between 25 and 55 years, and the occurrence is tal system, lungs, and heart, as well as arteritis and thick- three times more common in women than in men. In most ening of blood vessels. It affects the skin in 90% of pa- cases, RA begins insidiously, causing ill health and tients, the vascular system, esophagus, and intestines in chronic joint deformity. Rheumatoid factor is positive in 80%, lungs in 45%, heart in 40%, kidneys in 35%, and 50-70% of patients. Although the brunt of the disease oc- musculoskeletal system in 25% of patients. In addition, curs in the joints, extra-articular manifestations of this there is an association with primary biliary cirrhosis. The systemic connective tissue disease are present in up to clinical presentation includes fever, lassitude, and weight 76% of patients (Table 1). Therefore, RA is better termed loss. Antinuclear antibodies are present in 30-80% of pa- ‘rheumatoid disease’ [1-3, 6-8]. Indeed, 2-54% of pa- tients; anti-topoisomerase or antiScl-70 is present with tients with RA develop rheumatoid lung disease, which diffuse cutaneous involvement. Anti-centromere antibody occurs five times more often in men than in women. is present in a third of patients with scleroderma, and two-thirds of patients with CREST syndrome (calcinosis, Table 1. Raynaud’s, esophageal dysmotility, sclerodactyly, telang- Thoracic manifestations of rheumatoid arthritis iectasias) [1-4]. Pleura Effusion Thickening Radiologic Manifestations Pneumothorax Lungs Diffuse interstitial fibrosis Necrobiotic nodules (rheumatoid nodules) Basilar interstitial pneumonitis and fibrosis are the radi- Bronchial abnormalities ologic manifestations of scleroderma [3]. The basilar dis- Caplan syndrome tribution is more pronounced in scleroderma than in oth- Cardiovascular Cardiac enlargement er collagen vascular diseases. On radiographs there may Pulmonary arteritis be fine/coarse basilar reticulations with small lung vol- Osseous Erosive arthritis umes due to the restrictive process of the disease. High- Facet joint ankylosis resolution computed tomography (HRCT) demonstrates Rib erosions inter- and intralobular septal thickening, subpleural lines Osteoporosis and compression fractures 104_111_Kazerooni 28-02-2007 07:48 Pagina 105 Chest Manifestations of Systemic Diseases 105 Radiologic Manifestations Systemic Lupus Erythematosus Pleural abnormalities are the most frequent thoracic man- Demographics and Clinical Manifestation ifestation and include a unilateral exudative pleural effu- sion in 90% of patients (protein content >4 g/dl, a low Systemic lupus erythematosus has an incidence of 1 in glucose content of <30 mg/dl that does not rise during in- 2000, is usually diagnosed between 20 and 40 years of travenous glucose infusion), which may change little over age, and is nine times more common in women than in months. The fluid has a low white cell count, many lym- men. SLE is more prevalent in East Asia and especially phocytes, and is usually positive for rheumatoid factor, in the USA, where the incidence in African-American LDH, and RA cells [9]. Bilateral large effusions may al- women is as high as 1 in 250 and in whom the disease of- so be seen. Pleural disease is nine times more common in ten has a more severe course. There is an increased inci- men than in women and may antedate arthritis. In addi- dence of HLA B8 and DR3 antigens in SLE patients. The tion, pleural thickening may occur, usually bilaterally, disease may be exacerbated by sunlight and infection. with pleural fibrosis and adhesions being common find- Furthermore, a lupus-like syndrome may be induced by ings at autopsy. Pneumothorax may be present secondary certain medications, such as hydralazine, oral contracep- to rupture of rheumatoid nodules, which are often cavi- tives, phenothiazines, and procainamide. The most com- tary, or end-stage fibrotic lung disease. mon early features are fever, arthralgia, general ill health, Diffuse interstitial fibrosis is seen in 2-6% of patients and weight loss. The ‘butterfly’ rash on the face is char- and is most frequent in cases of seropositive RA [9]. acteristic. Antinuclear antibodies are present in 95% of This restrictive lung disease, with lower-lobe subpleural patients. Antibodies to double-stranded DNA are consid- predominance, is often indistinguishable from usual in- ered diagnostic, although Sm antibodies are more specif- terstitial pneumonitis (UIP form). As such, it manifests ic. Hypergammaglobulinemia occurs in 77% of patients, as irregular septal lines, honeycombing, or a reticulo- LE cells in 80%, and a false positive Wasserman test for nodular pattern. HRCT improves early detection. syphilis in 24%. SLE affects the joints in 90% of patients, Rheumatoid lung disease has a more benign course than skin in 80%, kidneys in 60%, lungs in 50%, cardiovascu- lar system in 40%, and nervous system in 35%. It also in- UIP [3, 9, 10]. volves the blood and lymphatic systems [1-3, 8]. SLE af- Necrobiotic nodules are well-circumscribed masses in fects the respiratory system more often than other con- the lungs, pericardium, and visceral organs. They are nective tissue diseases. identical pathologically to the subcutaneous nodules, and are seen with advanced disease. These nodules are usual- Radiologic Manifestations ly multiple, non-calcified, peripherally occurring, and range in size from 3 mm to 7 cm. They may cavitate, typ- Pleural effusion is the most common manifestation, with ically with a thick wall and smooth border. Necrobiotic recurrent small bilateral pleural effusions in 70% of pa- nodules are often found in conjunction with subcuta- tients. There may be pleural thickening. Pericardial effu- neous nodules that wax and wane [1, 3, 6, 9, 10]. sion from pericarditis is common, whereas cardiomegaly Bronchial abnormalities, such as bronchiectasis or bron- from primary lupus cardiomyopathy is rare [11, 12]. chiolitis obliterans, occasionally occur. Bronchiectasis is Acute lupus pneumonitis is characterized by ground- mainly seen in women with longstanding seropositive glass opacity/consolidation at the lung bases, evolving disease, while bronchiolitis obliterans is rare but fre- when chronic to basal interstitial pulmonary fibrosis in quently fatal. Follicular bronchiolitis is defined as hyper- approximately 3% of patients. Alveolar hemorrhage also plasia of BALT (bronchus-associated lymphoid tissue) manifests as ground-glass opacity or consolidation. and as such classified as a lymphoproliferative disorder. Bibasilar fleeting plate-like atelectasis secondary to in- Nonetheless, in two-thirds of cases it occurs in RA pa- fection or infarction may occur. Patients with SLE have tients. HRCT demonstrates nodular opacities 1-3 mm in an increased incidence of pulmonary emboli secondary to size (rarely up to 10 mm) in a centrilobular and peri- circulating antiphospholipid antibodies (Table 2). bronchovascular distribution. Pulmonary arteritis is a rare manifestation of RA. It consists of fibroelastoid intimal proliferation that may re- Table 2. Thoracic manifestations of systemic lupus erythematosus sult in pulmonary arterial hypertension and cor pul- Pulmonary Ground-glass opacity (pneumonitis or hem- monale [9]. Cardiopericardial silhouette enlargement is orrhage) also unusual, and may result from a pericardial effusion Consolidation (pneumonitis or hemorrhage) or myocarditis with congestive cardiac failure. Various Atelectasis bone abnormalities may be seen on chest X-ray or CT, in- Pulmonary embolism cluding erosive arthritis, ankylosis of vertebral facet Pleural Effusion (pleuritis) joints, osteoporosis, and vertebral-body collapse sec- Thickening ondary to steroid therapy. Caplan syndrome is the coex- Cardiovascular Pericardial effusion (pericarditis) istence of coal-workers pneumoconiosis and RA. Cardiomegaly (cardiomyopathy) 104_111_Kazerooni 28-02-2007 07:48 Pagina 106 106 E.A.Kazerooni Polymyositis/Dermatomyositis Radiologic Manifestations
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