Fibrobullous disease of the upper lobes: An extraskeletal manifestation of ankylosing spondylitis

From the Department of Radiology, New York University Medical Center, New York, New York, USA
Journal of Computed Tomography 08/1984; 8(3):225-9. DOI: 10.1016/0149-936X(84)90066-3
Source: PubMed


Fibrobullous disease of the upper lobes of the lungs is a rare extraskeletal manifestation of ankylosing spondylitis, occurring in 1.3% of patients with ankylosing spondylitis. We present a patient with this disease, and discuss this pulmonary manifestation. Because the radiographic appearance of the chest in this disease resembles that in tuberculosis, many patients are misdiagnosed and treated for tuberculosis despite negative bacteriology. Computed tomography is useful in delineating the extent of pleural thickening, bullous changes, volume loss, parenchymal fibrosis, and bronchiectasis, as well as identifying or excluding an intracavitary pulmonary mycetoma.

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    • "The incidence of apical fibrosis in ankylosing spondylitis is low with estimates ranging from 1.3–30% and has an association with longer disease duration [22]. Apical fibrosis often appears greater than five years after the start of the arthritic symptoms associated with the disease [23]. The fibrosis can be unilateral or bilateral in nature and is can be followed by cystic changes to the lung [24]. "
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    ABSTRACT: Ankylosing spondylitis is a chronic inflammatory condition that usually affects young men. Cardiac dysfunction and pulmonary disease are well-known and commonly reported extra-articular manifestation, associated with ankylosing spondylitis (AS). AS has also been reported to be specifically associated with aortitis, aortic valve diseases, conduction disturbances, cardiomyopathy and ischemic heart disease. The pulmonary manifestations of the disease include fibrosis of the upper lobes, interstitial lung disease, ventilatory impairment due to chest wall restriction, sleep apnea, and spontaneous pneumothorax. They are many reports detailing pathophysiology, hypothesized mechanisms leading to these derangements, and estimated prevalence of such findings in the AS populations. At this time, there are no clear guidelines regarding a stepwise approach to screen these patients for cardiovascular and pulmonary complications.
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    ABSTRACT: Chest radiographs of 39 patients with ankylosing spondylitis were studied. Three showed apical pulmonary fibrosis, two with cavitary lesions. Other known causes of lung disease were excluded. Symptoms and roentgenographic evidence of spondylitis were present for many years prior to the onset of pulmonary symptoms, which variably included shortness of breath, cough, hemoptysis, pleuritic chest pain, fever, and chills. Apical pulmonary lesions of unknown cause were absent in 53 age, sex, and racematched osteoarthritis control patients. The findings suggest that apical pulmonary fibrosis may be an extra-skeletal manifestation of ankylosing spondylitis, the frequency of which approaches that of spondylitic heart disease.
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