ReviewPleuropulmonary involvement in ankylosing spondylitis
Introduction
Ankylosing spondylitis (AS) is the archetypal spondyloarthropathy and as such is strongly associated with the HLA B27 antigen. The disease primarily affects the axial joints [1], most notably the sacroiliac joints. Extraarticular manifestations are represented by uveitis, aortic regurgitation, bowel disease, and other systemic disorders [2], [3], [4]. Respiratory abnormalities have been reported in 0% to more than 30% of patients with AS [5], [6], [7], [8]. Chest wall rigidity related to involvement of the costovertebral joints is responsible for a restrictive ventilatory pattern. In addition, AS can cause pleuropulmonary lesions, of which the most common are apical fibrosis, interstitial infiltrates, and pleural thickening [9], [10], [11]. These lesions are usually asymptomatic, and in their early stages they are not visible on chest radiographs. As a result, pleuropulmonary lesions were long believed to be uncommon and delayed manifestations of AS. However, over the last decade high-resolution computed tomography (HRCT) has been found superior over plain radiography in detecting early pulmonary lesions due to various systemic diseases including rheumatoid arthritis, systemic sclerosis, and AS [12], [13], [14], [15]. In patients with AS, HRCT has shown not only the classic lung lesions, but also a wide variety of subtle abnormalities undetectable by plain radiography. The relevance of these abnormalities remains to be determined.
This review article starts with a description of the steps that led to the recognition of pleuropulmonary abnormalities associated with AS. Then, the frequency and distribution of the abnormalities are discussed, with emphasis on differences related to the detection methods used (plain radiographs, HRCT, lung function tests (LFTs), bronchoalveolar lavage (BAL), and histological studies of transbronchial biopsy or autopsy specimens). The review ends with a presentation of current pathophysiological hypotheses and of the impact of pleuropulmonary manifestations on the everyday management of patients with AS.
Section snippets
Historical overview
The first report of pleuropulmonary disease in patients with AS was written in 1941 by Dunham and Kautz [16], who described four cases of apical tuberculosis among 20 patients with AS. From a literature review, they concluded that pulmonary tuberculosis was more common among AS patients than in the population at large. In 1949, Hamilton [17] also reported an excess risk of pulmonary tuberculosis in AS patients and suggested that this finding might reflect diagnostic error. Two of his patients
Chest radiographs
Rates of pleuropulmonary involvement were low in studies that used chest radiographs as the only imaging method. In the largest case-series, the rate of occurrence was 1.2% [25]. The only abnormalities detected in these studies were apical fibrosis, pleural thickening, bronchiectasis, and interstitial disease. Apical fibrosis develops in the upper lung lobes and follows a progressive course. Pulmonary fibrosis associated with AS is consistently located in the upper part of the lungs, in sharp
High-resolution computed tomography
Falashi et al. [36] wrote the first description of early pulmonary lesions detected by HRCT, in four patients with AS. Since then, including the report of our preliminary findings [35], only five prospective studies of pleuropulmonary lesions detected by HRCT in AS patients have been published. Table 2 recapitulates the HRCT changes found in these studies. Casserly et al. and Fenlon et al. [37], [38] reported HRCT abnormalities in 69% of 26 AS patients, of whom only 15% had abnormal chest
Lung function testing
AS is associated with a pure restrictive pattern due to rigidity of the chest wall [43], [44]. Vital capacity (VC) is decreased in proportion to disease severity; in patients with advanced diseases, VC is rarely less than 60% of predicted. Residual volume (RV) and functional residual capacity (FRC) are increased or normal, in contrast to findings in other restrictive diseases. Although expiratory muscle performance is enhanced, the chest wall rigidity causes muscle exhaustion, so that complete
Bronchoalveolar lavage
To elucidate the pathophysiological mechanism underlying pulmonary lesions in patients with AS, Wendling et al. [47] performed BAL in 15 patients and 17 controls. Two patients had interstitial disease in the lung bases; no information is given on the pulmonary status in the remaining 13 patients. BAL showed no evidence of inflammation, in striking contrast to experience with rheumatoid arthritis. In a study of 14 AS patients, Kchir et al. [48] found that BAL was normal in 12 patients and showed
Histological studies
Histological studies have been done on transbronchial or percutaneous biopsies, operative specimens, and autopsy specimens [51], [52]. Nonspecific interstitial fibrosis with degeneration of collagen and elastic fibers was found. Focal lymphocytic infiltrates were visible in some cases. In patients with advanced disease, cystic cavities were visible within the fibrous areas. No granulomas or signs of vasculitis were found. Kchir et al. [48] obtained transbronchial biopsies from 12 of their 14 AS
Etiological and pathogenic hypotheses
More than 50 years after the seminal description, pleuropulmonary involvement by AS remains an enigma. Pathophysiological hypotheses are based on two main theories, the mechanical theory and the AS-specific theory.
Evaluation of pulmonary involvement in clinical practice
A careful physical examination is crucial to the evaluation of the lungs in patients with AS. Chest expansion should be measured, and finger clubbing or other signs denoting hypoventilation should be sought. In patients who are free of symptoms, auscultation of the lung fields may reveal crackling rales that suggest fibrosis and indicate a need for further investigations. LFTs should be obtained to look for a restrictive pattern, most notably in patients with severe or long-lasting AS. A chest
Treatment and follow-up
The presence of pleuropulmonary abnormalities in a patient with AS has no impact on the treatment strategy. Prophylactic measures including smoking cessation and physiotherapy to improve overall and respiratory function may be useful, however, in conjunction with disease-modifying antirheumatic drugs and nonsteroidal antiinflammatory agents. Whether early and appropriate treatment of AS decreases the risk of pleuropulmonary involvement is not known. Longitudinal studies of patients with
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