A 64-year-old man was referred to our hospital with a diagnosis of pulmonary upper lobe fibrosis. He had experienced repeated pneumothorax since the age of 18 years. In his 30s, he noticed cough and mild fever. He received antituberculous medical therapy under the diagnosis of suspected pulmonary tuberculosis, but the cough remained. A few years ago, he noticed exertional dyspnea. Since then, cough and dyspnea have been gradually worsening and he has experienced repeated episodes of fever. He had smoked 20 to 30 cigarettes a day for about 20 years and had quit smoking in his late 30s. His mother had rheumatoid arthritis and systemic sclerosis.
On admission, he was slender with a BMI of 17.9 kg/m2 and had no clubbed fingers. Respiratory sounds were normal. A chest radiograph showed upper lobe-dominant fibrotic opacities with elevated hilar opacities (Fig. 2a). Chest CT showed subpleural consolidations with traction bronchiectasis and bilateral apical cysts (Fig. 2b). However, the lower lung fields were almost free from fibrotic lesions. FVC and total lung capacity (TLC) were decreased: 2220 mL (59.4% pred.) and 4970 mL (87.0% pred.), respectively. However, residual volume (RV) was increased (2850 mL, 142.5% pred.), which resulted in an increased RV/TLC ratio (154.6% pred.).
He underwent fiber-optic bronchoscopy examination. TBLB specimens obtained from the left upper lobe showed fibrosis, but there was no specific diagnosis. However, the histological specimen stained with EVG revealed dense aggregates of elastic fibers that were associated with intra-alveolar fibrosis (Fig. 2c, d). These histological findings are consistent with those of PPFE.
b. Upper lung fields of chest CT in case 2.