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Imaging of the Pulmonary Manifestations of Systemic Disease Postgrad Med J 2001;77:621–638 621 REVIEWS Postgrad Med J: first published as 10.1136/pmj.77.912.621 on 1 October 2001. Downloaded from Imaging of the pulmonary manifestations of systemic disease A G Rockall, D Rickards, P J Shaw Lung involvement in systemic disease may be a manifestation of the underlying pathological Box 1: HRCT signs (adapted from process, may be a complication of the under- Webb et al223 p 118, 207, 243) lying disease or may be related to the Fibrosing alveolitis treatment. Lung pathology is dominant in cer- 1. Findings of fibrosis: intralobular tain diseases, such as in Wegener’s granuloma- interstitial thickening, irregular interfaces, tosis, but may be only rarely present, for exam- visible intralobular bronchioles, ple in Henoch-Schönlein purpura. However, honeycombing, traction bronchiectasis.* lung involvement has a profound eVect on 2. Irregular interlobular septal thickening. prognosis and may be challenging to accurately 3. Ground glass opacity. diagnose. In some patients, bronchoalveolar 4. Peripheral and subpleural predominance lavage and tissue diagnosis with transbronchial of abnormalities.*† or percutaneous biopsy is not possible, due to 5. Lower lung zone and posterior the poor clinical state of the patient. predominance.*† Imaging often plays a central part when lung Bronchiectasis involvement is suspected clinically and this role 1. Bronchial dilatation.*† has increased with the advent of high resolution 2. Bronchial wall thickening.*† computed tomography (HRCT). The chest 3. Visibility of peripheral airways.*† Department of radiograph may provide diagnostic information 4. Contour abnormalities *†—for example, Radiology, University and be useful in follow up but it is relatively signet ring (vertically orientated bronchi), College London insensitive. HRCT now has several established tram tracks (horizontally orientated Hospitals, London, UK roles: bronchi), loss of tapering. A G Rockall (1) May be diagnostic and if not will often 5. Fluid filled bronchi.*† D Rickards narrow the diVerential diagnosis.12 This in P J Shaw 6. Atelectasis. turn may reduce the need for biopsy.3 The Bronchiolitis obliterans organising pneumonia HRCT signs of interstitial lung disease, small Correspondence to: (BOOP) Dr A Rockall, airways disease and bronchiectasis are well 1. Patchy bilateral airspace consolidation.* http://pmj.bmj.com/ Department of Academic established (see box 1). Radiology, St Bartholomew’s 2. Ground glass opacity.* (2) May demonstrate pathology when the Hospital, Dominion House, 3. Subpleural and/or peribronchovascular St Bartholomew’s Close, chest radiograph appears normal, in patients distribution.* London EC1A 7BE, UK with respiratory symptoms or abnormal pul- 4. Bronchial wall thickening, dilatation in monary function tests. This particularly applies Submitted 15 May 2000 abnormal areas.* to diseases in which the radiographic signs are Accepted 6 March 2001 5. Small nodular opacities, often subtle or obscured by overlying structures, for peribronchiolar. Outline example obliterative bronchiolitis, bronchiecta- Section A: connective tissue 6. Combination of findings 1 and 2.*† on October 1, 2021 by guest. Protected copyright. diseases sis, early fibrosing alveolitis, and fine walled Rheumatoid arthritis cystic structures, such as in lymphangioleio- Obliterative bronchiolitis Systemic lupus erythematosis myomatosis. 1. Areas of decreased lung opacity, patchy Sjogren’s syndrome in distribution.* Polymyositis/dermatomyositis (3) Assessment of disease activity. Several Progressive systemic sclerosis studies suggest that ground glass shadowing on 2. Bronchiectasis.* Mixed connective tissue HRCT in fibrosing alveolitis corresponds 3. Attenuation of pulmonary vessels.* disease 4 4. Combination of 1–3.† Ankylosing spondylitis histologically to active alveolitis. This in turn 5 Relapsing polychondritis predicts a better response to treatment and 5. Areas of consolidation or increased lung better prognosis.6 Although ground glass shad- opacity. Section B: systemic vasculitides owing is non-specific, it often represents 6. Reticulonodular opacities. Classification Small vessel vasculitis reversible pathology, such as infection, haemor- *Most common findings; †findings most helpful in Medium and large vessel rhage, or oedema. diVerential diagnosis vasculitides (4) Assessment of interval change and treat- DiVuse alveolar haemorrhage ment response, by acquiring comparative scans Section C: miscellaneous on follow up. (7) Prospective HRCTstudies may help in Lysosomal storage diseases (5) Prognostic information. understanding the natural history of lung Amyloidosis (6) Planning a biopsy: for example trans- involvement in systemic disease. Langerhans cell histiocytosis Erdheim-Chester disease bronchial biopsy in peribronchial disease or Recently, several groups have published Primary ciliary dyskinesia percutaneous in subpleural disease and in HRCT findings in several of the systemic Inflammatory bowel disease guiding the optimal site for open biopsy, by diseases. This evidence based article reviews Neurofibromatosis Tuberous sclerosis/ defining areas of active alveolitis and avoiding the radiological features of lung involvement, lymphangioleiomyomatosis areas of established fibrosis. including the recent literature on HRCT www.postgradmedj.com 622 Rockall, Rickards, Shaw Postgrad Med J: first published as 10.1136/pmj.77.912.621 on 1 October 2001. Downloaded from Box 2: Pleuropulmonary manifestations of rheumatoid arthritis Pleural x Pleuritis/pleural thickening.* x Pleural eVusion.* x Empyema. x Pneumothorax. Parenchymal x Interstitial lung disease.* x Nodules. x Caplan’s syndrome. Airways x Bronchiectasis.* x Bronchiolitis obliterans organising pneu- monia (BOOP). Figure 1 Chest radiograph of a 60 year old man, with x Obliterative bronchiolitis. rheumatoid arthritis and progressive dyspnoea, showing signs of fibrosing alveolitis with basal volume loss and x Bronchocentric granulomatosis. reticular opacities, more pronounced on the right (courtesy x Follicular bronchiolitis. of Dr H Booth). Pulmonary vasculitis/hypertension Drug induced lung disease pulmonary function test abnormalities in Amyloidosis 40%11 22; and histological changes in 80% of *Most common findings patients, including some asymptomatic pa- tients with a normal chest radiograph.23 There is a male preponderance (2M:1F) appearances, in the connective tissue diseases, with an insidious onset in the 50s, with a cough systemic vasculitides and miscellaneous sys- and/or dyspnoea. Patients are usually seropos- temic diseases with lung involvement. itive, with established joint disease in 90%, and have subcutaneous nodules and finger club- *** bing.11 Over 70% of patients are smokers. The appearances on chest radiography are Section A: connective tissue diseases indistinguishable from cryptogenic fibrosing RHEUMATOID ARTHRITIS alveolitis, with bibasal reticular, reticulonodu- The lungs, heart, and the vascular endothelium lar, or honeycomb interstitial opacities and may be involved in rheumatoid arthritis. There progressive volume loss but may be asymmetric is a strong association with a positive rheuma- (fig 1).12 21 Pleural abnormalities and pulmo- toid factor when systemic manifestations or nary nodules, if present, may help to distin- vasculitis are present. Pulmonary involvement guish RA-ILD from cryptogenic fibrosing (box 2) is significant prognostically. In a large alveolitis.11 autopsy study, Tyoshina et al reported that lung HRCT demonstrates interstitial lung disease http://pmj.bmj.com/ involvement was second to infection as the 7 in patients with and without clinical evidence most common cause of death (18% v 27%). of the disease (69%–80% and 20%–29%).21 24 The signs are those of cryptogenic fibrosing Pleural disease alveolitis (fig 2, box 2).18 21 25 Follow up HRCT Pleural disease is common in postmortem demonstrates progressive honeycombing from 89 studies (40%–75%) and is associated with the lung bases towards the apices.21 Emphy- subcutaneous nodules, interstitial lung disease sema and bronchiectasis have been reported in on October 1, 2021 by guest. Protected copyright. and pericarditis, in middle aged men with high association with RA-ILD, including non- 9–11 rheumatoid factor titres. EVusions, seen in smoking patients.24–26 912 3%–5%, usually occur at periods of active DiVuse interstitial pulmonary amyloidosis 13 arthritis but may precede the arthritis. They may mimic interstitial lung disease and should 14 are usually small, unilateral and asympto- be considered in the diVerential diagnosis in 15 matic, with mild pain in 20%–28%. They cases with longstanding rheumatoid arthritis.27 often resolve over weeks but may be persistent Computed tomography is used to direct 16 and recurrent. Pleural thickening is seen on biopsy towards areas of presumed active alveo- 17 chest radiography in 20%. Analysis of the litis (ground glass areas). Histology is often 18 pleural fluid may be helpful diagnostically. mixed, including interstitial pneumonitis, Pneumothorax and empyema are unusual bronchiolitis obliterans organising pneumonia 19 20 findings and may be secondary to cavitation (BOOP), lymphocytic interstitial pneumonitis, of a necrobiotic nodule. A spontaneous sterile lymphoid hyperplasia, and rheumatoid nod- empyema may develop during active rheuma- ules.18 The features are similar to cryptogenic 19 toid arthritis. fibrosing alveolitis except for an increase in lymphoid follicles, which is suggestive of Parenchymal disease RA-ILD or the presence of rheumatoid
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