Rapidly Progressive Pulmonary Apical Fibrosis and Parenchymal Destruction in a Patient with Ankylosing Spondylitis

Abstract

Pulmonary apical fibrosis is a rare complication of ankylosing spondylitis (AS). The essential characteristics of this lesion are its very slow progression and frequently asymptomatic nature. Herein, we are presenting a patient with AS who rapidly developed pulmonary apical fibrosis in a 3-year period despite decreased musculoskeletal pains. The 60-year-old male applied with complaints of progressively increasing cough in the recent two years, dyspnea, and fatigue. He had no chronic disease except AS. He had no continuous medication except nonsteroid anti-inflammatory drugs for 2-3 days monthly since his musculoskeletal pains decreased in the recent years. His physical examination revealed reduced breath sounds in the upper zones of the right lung. Chest X-ray revealed increased diffuse opacity in the upper zones of the right lung. Thoracic high-resolution computed tomography showed a consolidation accompanied with traction bronchiectases compatible with chronic fibrosis in the upper lobe of the right lung. However, thoracic computed tomography of the patient performed 3 years ago did not reveal pulmonary apical fibrosis and parenchymal destruction. Biopsy revealed no finding of malignancy, granulomatous inflammation, or vasculitis. The results of cultures were negative. So, the patient was diagnosed as pulmonary involvement of AS, which developed in a 3-year period. This case has shown that extra-articular complications may continue to develop in patients with AS even if their musculoskeletal complaints have subsided. So, patients with AS should be followed up regularly with systemic examinations.

Apical fibrobullous lung disease in ankylosing spondylitis: case report and literature review

“…These may include pleuro-parenchymal changes in 40–90% of patients with AS in early phases (less than 10 years of disease) [ 7 , 8 ]. Besides AFBD, with or without cavitation, these may include findings of pleural thickening, bronchiectasis, emphysema, small airways disease, ground-glass opacities and pleural effusion [ 5 , 7 , 9 ]. Non-specific parenchymal linear opacities have also been described in patients with AS with no respiratory symptoms, probably translating early lung disease [ 10 ].…”

“…Some have proposed that extra-articular AS manifestations parallel the inflammatory process responsible for the articular ones [ 4 ]. In lung biopsies, cellular inflammatory infiltrates and interstitial fibrosis have been found [ 9 ]. A rough joint inflammation control conditioning daily symptoms and frequent use of rescue medication, as in the described case report, may have contributed to the AFBD development.…”

“…It usually shows slow progression. However, a great variability exists, and pulmonary lesions may occur 6–35 years after first joint manifestations, in asymptomatic patients as well as in early AS [ 1 , 8 , 9 ]. In the presented case, there was a 17-year evolution AS, but the lack of a chest CT before first respiratory evaluation at our department unenabled time estimation of lung involvement.…”

Imaging of Lung Disease Associated with Connective Tissue Disease

“…192,194 Ordinarily, this is slowly developing over several years, although a recent case report documented rapidly progressing apical fibrosis in a matter of 3 years. 195 Patients are prone to superadded fungal or mycobacterial disease, 191,193 and spontaneous pneumothorax, which may be recurrent and bilateral. 196,197 The advent of HRCT allowed for the fuller assessment of the pleuroparenchymal and airway disease associated with AS.…”