CASE REPORT: BI-APICAL PULMONARY FIBROSIS WITH BRONCHIECTASIS IN A PATIENT WITH ANKYLOSING SPONDYLITIS

Abstract

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Ankylosing spondylitis (AS) is a chronic inflammatory disease primarily affecting joints of the axial skeleton. In addition to disabling joint changes, pulmonary manifestations beyond the mechanical restrictive syndrome imposed by costovertebral joint ankylosis have been described [1]. These changes are seen on chest X-ray (CXR), pulmonary function tests (PFTs), bronchoalveolar lavage (BAL), and high-resolution chest tomography (HRCT) in varying degrees. They do not correlate with severity and duration of the primary disease [2]. Of the pulmonary findings, apical fibrosis is among the most distinctive. CASE PRESENTATION: We report a case of a 56-year-old male with long-standing AS (on indomethacin) who developed a dry cough with intermittent dyspnea and lung changes that were followed radiographically for several years. An initial CXR obtained for unrelated pre-operative purposes was unremarkable 21 years into his AS disease course. Two years after the first CXR, a right apical mass-like density with associated pleural thickening was detected. A QuantiFERON test was negative. Further evaluation by a computed tomography (CT) scan of the chest demonstrated volume loss of the right upper lobe with associated ground-glass opacities and pleural thickening. PFTs were consistent with a restrictive pattern, though the results were technically confounded by cough. Three years later, a follow-up CT scan of the chest showed significant progression to severe bi-apical fibrosis of the lungs with bronchiectasis and pleural thickening. Throughout this time, his joint disease remained stable, but his chronic cough worsened to the point of post-tussive emesis. DISCUSSION: The literature reports that a patient with AS with a normal CXR, normal PFTs, and lack of respiratory symptoms can still have pulmonary manifestations detectable by HRCT [3]. This patient’s cough first started 12 years before his first abnormal CXR. Despite his initial normal CXR, it is likely that radiographic evidence of the pulmonary changes predates this if evaluated by advanced chest imaging modalities. The pathogenesis of these pulmonary complications remains unknown; however, studies of BAL fluid have shown that subclinical inflammation of the alveoli is not a contributor [2-3]. CONCLUSIONS: The clinical significance of these early pulmonary changes is not known. There is a lack of studies evaluating benefit in obtaining advanced chest imaging on asymptomatic patients with AS. These changes were thought to be rare; however, newer studies suggest a higher prevalence. For patients presenting with respiratory symptoms with inflammatory arthritis, it is important to consider pleuropulmonary changes (ranging from airway disease to parenchymal scarring) in setting of AS. Reference #1: Kiris A, Ozgocmen S, Kocakoc E, Ardicoglu O, Ogur E. Lung findings on high resolution CT in early ankylosing spondylitis. Eur J Radiol. 2003;47(1):71-76. Reference #2: Kchir MM, Mtimet S, Kochbati S, et al. Bronchoalveolar lavage and transbronchial biopsy in spondyloarthropathies. J Rheumatol. 1992;19(6):913-916. Reference #3: Quismorio FP. Pulmonary involvement in ankylosing spondylitis. Curr Opin Pulm Med. 2006;12(5):342-345. https://doi.org/10.1097/01.mcp.0000239551.47702.f4 DISCLOSURES: No relevant relationships by Sudhir Rajan, source=Web Response No relevant relationships by Eduardo Solbes, source=Web Response No relevant relationships by Paulina Sun, source=Web Response