A 47-year-old man presented with complaints of cough and progressive breathlessness of 2 months' duration. There was fever, malaise, anorexia, and weight loss. Auscultation revealed crackles in the right mammary region. Rest of the physical examination was unremarkable. Complete blood count, fasting plasma glucose, liver and renal function tests were all within normal limits. Chest radiograph showed bilateral hilar enlargement and nonhomogenous opacities in the mid and lower zones of both lungs along with cavitation in the right middle zone. Sputum examination for acid-fast bacilli performed on 3 consecutive days was negative. The patient received empiric antituberculosis treatment with isoniazid, rifampicin, ethambutol, and pyrazinamide for a period of 2 months, followed by isoniazid and rifampicin for 4 months. Despite good compliance with treatment, cough and dyspnea worsened over the course of treatment. Chest radiograph after 6 months of therapy showed bilateral hilar adenopathy, reticulonodular opacities in both upper zones, bilateral perihilar consolidation, and a cavity in the right middle lobe [Figure 1A]. The patient was referred to us with a suspicion of drug-resistant tuberculosis.
Computed tomography (CT) of the chest showed enlarged intrathoracic lymph nodes with bilateral perihilar consolidation and cavitation in both the lower lobes [Figure 1B]. There were extensive peribronchovascular nodules and subpleural nodules. Tuberculin skin test performed with 5 tuberculin units was negative and serum angiotensin-converting enzyme levels were 110 U/L (normal 8-65 U/L). Spirometry showed moderate restrictive defect (forced vital capacity [FVC], 50% predicted). Diffusion capacity for carbon monoxide was decreased (2.74 mmol/min/kPa; 32% predicted). Flexible bronchoscopy revealed widespread fine nodularity in the entire bronchial tree. Transbronchial lung biopsy and endobronchial biopsy showed compact noncaseating epitheloid granulomas; stain for acid-fast bacilli or fungi revealed no organisms. Bronchoalveolar lavage fluid was negative for fungus, Mycobacterium tuberculosis (culture and nucleic acid amplification assay) and malignant cells. A diagnosis of PCS was made.
The patient was treated with oral glucocorticoids for 9 months. There was improvement in dyspnea and exercise capacity. CT performed 1 year after stopping treatment showed clearing of perihilar consolidation and peribronchovascular nodules. Furthermore, there was resolution of the cavity on the left side; however, a thin-walled cavity persisted in the right lower lobe [Figure 1C]. There was improvement in lung function (FVC, 65% predicted) and diffusion capacity (68% predicted). The patient remains stable on follow-up without any relapse or complication.