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

ABSTRACT 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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    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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