Forty-seven-year-old, male, ex-smoker (30 pack-year), diagnosed with AS HLA B27 + at the age of 30. He had, for several years, symptoms of cervical morning stiffness and axial pain of inflammatory rhythm, conditioning frequent use of non-steroidal anti-inflammatory medication, bilateral sacroiliitis grade IV, lumbar spine ankylosis and syndesmophytes as well as a restrictive ventilatory pattern. He had been medicated with Infliximab for almost 2 years, after negative screening for latent TB (4.5 years prior to first contact with the Pulmonary Department), was no longer under biological therapy and had abandoned ambulatory follow-up in Rheumatology 3 years ago.
He was admitted at the Pulmonary Department of a District Hospital with a 3-month evolution worsening dyspnea, productive cough with mucopurulent sputum, evening sweating and fever, anorexia, and unquantified weight loss. On initial physical examination, there was low body mass index (BMI <18.5 kg/m2), muscle wasting, pronounced thoracic kyphosis, decreased chest wall expansion, and crepitant rales over the upper thirds of both hemithorax. Laboratory study revealed elevated erythrocyte sedimentation rate (120 mm/h) and C-reactive protein (CRP 23 mg/dL), anemia (hemoglobin (Hb) 10.3 g/dL), and hypoalbuminemia (albumin 3.1 g/dL). Chest x-ray (Figure 1A) showed hypotransparency of the upper thirds of both pulmonary hemifields with retractile findings and lung volume loss as well as a cavity in the right upper lobe (RUL). Chest CT confirmed RUL consolidation with cavitation; left upper lobe (LUL) peripheral consolidation with air bronchogram; important upper lobe (UL) volume loss and multiple cylindric bronchiectasis (Figure 1 B and C). Sputum molecular tests detected Mycobacterium tuberculosis complex (MTC) and first-line anti-bacillary therapy was initiated. Since fever and cough persisted, a flexible bronchoscopy was performed, and a methicillin-resistant Staphylococcus aureus (MRSA) was isolated on bronchial biopsies. The patient was treated according to drug-susceptibility test, with improvement. On the seventh month of anti-bacillary therapy, he developed persistent dry cough. A new chest CT was performed, which revealed retractile AFBD, with significant lung volume loss and bronchiectasis as well as a bulky RUL-cavitated lesion with mediastinal contact. 
Flexible bronchoscopy with bronchial wash and biopsies was repeated, with no microbiological isolation or characteristic histopathological findings. The diagnoses of progressive AFBD secondary to AS aggravated by TB sequelae were assumed. The patient was referred for physical and pulmonary rehabilitation, re-evaluation by a rheumatologist (after patients' consent and willingness) and evaluation by a thoracic surgeon. However, 9 months later (16 months after first admission), the patient worsened, and was admitted again at the Pulmonology Department, presenting a 1-month evolution worsening of productive cough, increased purulent sputum, hemoptysis, and weight loss. On physical examination, he had worsening muscle wasting, low oxygen saturation on pulse oximetry and bilateral crepitant rales. Blood tests revealed microcytic hypochromic anemia (Hb 9.2 g/dL), leukocytosis 18.100 with neutrophilia 93% and elevated CRP 18.7 mg/dL. He had worsened chest x-ray findings with a significant wider cavitation in the RUL and bilateral reticular infiltrates at the right lower lobe and LUL (Figure 2A). Empirical antibiotic therapy was initiated. Revaluation by chest CT revealed size increase of RUL cavitation, condensation with air bronchogram particularly at the ULs, mass-like lesion inside LUL cavitation, diffuse multifocal ground-glass opacities, sub-pleural micronodules, and tree-in-bud (Figure 2 B and C). Sputum microbiology was inconclusive. A new flexible bronchoscopy was performed and Aspergillus fumigatus (AF) was isolated in the bronchial wash with an elevated bronchial wash Galactomannan antigen test and elevated serum AF IgG levels. He initiated voriconazole for the treatment of CCPA. During hospitalization, he presented fast worsening of respiratory failure and severe acute hemoptysis with a sudden 3 g/dL drop of Hb and the evidence of a new chest x-ray finding of an air-fluid level at the RUL cavity (Figure 2D). Poor performance status conditioned by low BMI, sarcopenia and extent of pulmonary and extra-pulmonary disease determined unfitness for surgical intervention and other major invasive procedures. The patient worsened fast and died despite optimized medical treatment before arterial embolization could be attempted.
Chest CT and x-ray during first admission to Pulmonary Department. Chest x-ray depicting right upper lobe (RUL) cavity, retractile findings, and bilateral lung volume loss (a).