Case Presentation

A 40-year-old female presented with 7 years of intractable cough and shortness of breath. There was no significant exposure history. She was a non-smoker and suffered from grass and dust mite allergy. On examination, she had finger clubbing and end-inspiratory fine crackles at both lung bases. A chest CT showed evidence of ground glass opacities, interlobular septal thickening, and focal areas of air trapping in both lung bases, in line with interstitial lung disease suggestive of hypersensitivity pneumonitis. A complex lung function test showed restrictive lung disease with a forced vital capacity of 1.65 L (45% of predicted value) and a total lung capacity of 2.7 L (56% of predicted value). A left-sided video-assisted thoracotomy lung biopsy in late 2017 showed extensive scarring and honeycombing with peribronchial fibrosis and granulomata, which confirmed her diagnosis of hypersensitivity pneumonitis. Her symptoms worsened in early 2018, and she developed a type 1 respiratory failure requiring oxygen. She underwent pulse therapy with an intravenous injection of 1 g of cyclophosphamide and methylprednisolone, and treatment continued with a weaning dose of oral prednisolone. Unfortunately, there was no response to this treatment course, and she underwent bilateral sequential lung transplantation in December 2018.