A 57-year-old woman presented in the emergency department complaining of progressive dyspnea for the previous 5 days. On clinical examination she had productive cough with clear sputum and intermittent fever. She was a lifelong nonsmoker but with multiple hospitalizations due to respiratory infections and asthma-like episodes from the age of 8 years. During the past 15 years the patient was treated symptomatically with antibiotics, nebulizer bronchodilators, and long-term oxygen therapy (2 L/min/24 hours). She had daily exposure to gases from a brazier used at home since childhood. Her family past medical history included her 30-year-old sister's sudden death from unknown reasons and her mother's infection from tuberculosis.
At the time of admission her axillary temperature was 38 C and blood pressure 120/70 mmHg. The patient was in respiratory insufficiency with an arterial pH of 7.46, PCO2 of 56 mmHg, PO2 of 72 mmHg, and HCO3 of 30 mmol/L on air. Lung auscultation revealed widespread bilateral crepitating and crackles over the lower part of both lungs. No lymphadenopathy was found and examination of other systems was unremarkable. The electrocardiogram was normal with a heart rate of 69 beats per minute.
Laboratory data showed an elevated erythrocyte sedimentation rate of 100 mm/h and a white blood count of 9000/muL without peripheral blood eosinophilia, and C-reactive protein was 9.3 mg/dL. Biochemical values were normal. Her sputum sample for acid-fast bacilli was negative on multiple occasions. Sputum sent for fungal culture did not grow any organisms. Screening for Aspergillus in sputum cultivation was also negative. Pseudomonas aeruginosa was isolated from her sputum culture. Urine antigen for Legionella and Streptococcus was negative.
Her chest X-ray demonstrated increased lung volumes and revealed multiple ring shadows in lungs that were almost symmetrical in distribution and more apparent in the mid and lower zones. There was the sense of air-fluid levels in some of them corresponding to cystic bronchiectasis. Also, there was thickening of the interstitial network. The trachea and main bronchi were normal (Figure 1).
A laboratory investigation was performed for alpha-1 antitrypsin level and was normal. Total serum immunoglobulin (Ig)E concentration was elevated at 2500 U/mL (normal range 0-165 U/mL). Immediate cutaneous reaction to Aspergillus fumigates was not available at the time of the patient's hospitalization; nevertheless, A. fumigatus-specific serum IgE levels were 1.37 U/mL. This is essential for the diagnosis of allergic bronchopulmonary aspergillosis.
Total serum IgG, IgA, and IgM levels were quantified to rule out hypogammaglobulinemia and did not reveal any deficiencies. Antinuclear and antineutrophilic cytoplasmic antibody tests were performed to exclude Churg-Strauss syndrome, and the results were negative.
A pulmonary function test demonstrated a mixed obstructive and restrictive pattern. There was a reduced forced vital capacity of 1200 mL (43.35% pred), reduced expiratory volume in 1 second of 720 mL (30.7% pred) with Tiffeneau index, reduced expiratory volume in 1 second/forced vital capacity of 76.29, and carbon monoxide-diffusing capacity of 28%.
A contrast-enhanced computed tomography (CT) scan of the chest demonstrated central cylindrical/cystic bronchiectasis involving segmental/subsegmental bronchi, bronchial wall thickening with air-fluid levels, and mucus plugging with predominant localization in the lower lobes. There was ground glass and tree-in-bud scattered in all lobes, a finding consistent with pneumonitis and acute bronchiolitis. Chest CT also revealed a mosaic picture, which also emerges in all lobe findings consistent with bronchiolitis obliterans. Collapse of bronchi with distal air trapping, the result of an excessively compliant bronchial wall, particularly in the left and right lower lobe, was also present (Figure 2).
We also performed fiberbronchoscopy on the patient. The findings were unremarkable. The bronchoalveolar lavage fluid obtained from the patient for an acid-fast bacillus was negative on multiple occasions, but the same P. aeruginosa was again isolated. The bronchoalveolar lavage fluid was negative for Aspergillus antibodies (IgG and IgM).
A diagnosis of saccular bronchiectasis due to Williams- Campbell syndrome was made based on clinicoradiological findings, laboratory testing, and past medical history. Genetic testing was planned, but the patient refused further investigations due to financial constraints. The patient was treated with intravenous antibiotics; third-generation cephalosporins, ciprofloxacin, and acetylcysteine; and chest physiotherapy, and was discharged after 1 month.
Written informed consent was obtained from the patient upon discharge for publication of this case report and all accompanying images.
Sequential axial high-resolution computed tomography images of the upper, middle, and lower lung fields demonstrate multiple large and coalescent cystic bronchiectases, associated with volume loss. Some of the bronchiectases have air-fluid levels that are due to brochial secretions.