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.
Ankylosing spondylitis (AS) is a chronic inflammatory disease, and it mainly affects the axial skeleton and peripheral joints [
The 60-year-old male patient came to the chest diseases department of our hospital with the complaints of progressively increasing cough, dyspnea, and fatigue in the recent two years. The patient who denied hemoptysis said that he rarely produced sputum. He had no chronic disease except AS diagnosed in 1976. He never smoked. He had no alcohol consumption. He had no continuous medication except nonsteroid anti-inflammatory drugs (NSAIDs) for 2-3 days monthly since his pain of the musculoskeletal system decreased dramatically in the recent years. His physical examination revealed a blood pressure of 110/70 mmHg and 36.8°C body temperature. Heart beats were rhythmic, and no murmur was present. Breath sounds decreased in the upper zone of the right lung. The chest expansion and the lumbar Schober test measured 0 cm. Laboratory test results were found as follows: erythrocyte sedimentation rate (ESR) 72 mm/h, C-reactive protein (CRP) 28 mg/dL (normal range: 0–5), leukocytes 7800/mm3, hemoglobin 11 g/dL, platelets 323.000/mm3, and total protein/albumin ratio 6.8/3.3 g/dL. Other biochemical tests, electrolytes, and urinalysis results were within normal limits. Spirometric pulmonary function tests (PFT) revealed a restrictive pattern: a forced vital capacity (FVC) of 1.58 L (38% of predicted), a forced expiratory volume in 1 s (FEV1) of 1.50 L (46% of predicted), and an FEV1/FVC 94% and carbon monoxide diffusion capacity (DLCO) of 23 mL/min per mmHg (43% of predicted). Lung radiography revealed increased diffuse opacity in the upper zones of the right lung (Figure
Chest X-ray shows diffuse opacities in the upper zone of the right lung. Also, it reveals deviation of the trachea to the right side and blunting of the right costophrenic sinus.
Thorax HRCT performed in 2019. It shows chronic consolidation with traction bronchiectasis compatible with fibrosis in the apical and posterior segments of the upper lobe which is extending to the superior segment of the lower lobe. Nodular alveolar densities in the lateral basal segment of the right lower lobe are also seen.
Thorax CT performed in 2016. There is no apical fibrotic changes and traction bronchiectasis in the upper zone of the right lung.
Pulmonary apical fibrosis is a rare extra-articular manifestation which is seen in the late stages of AS. The essential characteristics of this lesion which may be unilateral or bilateral are its very slow progression and frequently asymptomatic nature. The lesions may start to appear as linear or patchy opacities in the lung graphy and become larger by joining together in time. They may radiologically mimic tuberculosis and malignancy. Our case is important with respect to development of pulmonary apical fibrosis, parenchymal destruction, and various nonspecific interstitial abnormalities within three years despite musculoskeletal symptoms significantly reduced by years. The samples of transbronchial biopsy revealed no finding of malignancy, granulomatous inflammation, or vasculitis. The staining and culture tests from the obtained samples showed negative results. Also, PCR and IGRA tests performed for tuberculosis resulted negative. In the light of these results, the patient was diagnosed with pulmonary involvement of AS.
Pulmonary involvement was considered as a very rarely seen late-term complication of AS before the introduction of HRCT to the use. A retrospective analysis of lung radiographies from a large series of AS patients revealed 28 (1.3%) of 2080 patients, and most of those (25 patients) were reported to have upper lobe fibrosis. The studies which were conducted using HRCT in the later years have noted that various pleuroparenchymal manifestations were detected in 40–90% of the patients with AS [
In contrast with the conclusion in the previous years, abnormal HRCT findings are frequently (64–71%) encountered also in the early-stage AS patients (disease duration < 10 years) with normal lung graphy [
Since the causes of pulmonary parenchymal destruction in the AS patients are not yet clarified, no definitive treatment recommendation is available. It is considered that interstitial inflammation triggered by disease-specific mechanisms also plays a role, as well as chest cage rigidity. Chronic inflammatory cell infiltrates and prominent interstitial fibrosis have been reported in the biopsies [
Pulmonary apical fibrosis is a well-known extra-articular manifestation of AS, and it may be seen in the late stages of the disease. This manifestation is commonly asymptomatic and progresses very slowly in the years. However, the comparison between two separate CT imaging studies performed in our patient with an interval of three years revealed the development of rapidly progressive pulmonary apical fibrosis and parenchymal destruction. Because serious extra-articular complications may develop, patients with AS should be followed up regularly with systemic examinations even if their complaints have subsided.
All data in this case report are taken from the clinical records.
The authors declare that they have no conflicts of interest.