title: Fatal hypersensitivity pneumonitis.
abstract: BACKGROUND
  Hypersensitivity pneumonitis (HP) is an uncommon, non-IgE-mediated interstitial lung disease caused by the inhalation of a variety of organic dusts, most commonly from exposure at work or in the pursuit of hobbies. Typically, after the disease is recognized, the causative allergen or environment is identified and treatment initiated through avoidance measures and corticosteroids. Progression of the disease is then usually halted and even reversed. Fatal cases of HP are unusual.
  
  
  OBJECTIVE
  To report a case of progressive and deadly HP in a 40-year-old printer who developed subacute bird fancier's disease with its clinical characteristics and positive precipitins to pigeon proteins.
  
  
  METHODS
  Chest x-ray examinations and tests of lung function were performed in the patient. Two months after initial consultation, when the diagnosis was still elusive, an open lung biopsy was performed and the patient was treated with prednisone for 3 months. A subsequent chest x-ray examination was performed 4 months after the biopsy.
  
  
  RESULTS
  With avoidance of birds and treatment with corticosteroids, the patient's symptoms resolved and lung function normalized. He was subsequently diagnosed as having asthma followed by bronchitis and 2 episodes of pneumonia. He did not fully recover from these but developed progressive dyspnea. After linking his symptoms to work by history, he underwent lung biopsy with findings consistent with chronic HP. Serum antibody titers were positive for Aspergillus but not pigeon proteins. Based on exposure to water-based coolants, he was suspected of having chronic occupational HP, although this could not be confirmed. Despite aggressive treatment, he developed a progressive course that was ultimately fatal.
  
  
  CONCLUSIONS
  This report details the progressive disease course in an individual who presented initially with subacute HP. Unfortunately, even after appropriate diagnosis and management, the course of the disease can be fatal.
authors.affiliation: Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin 53201, USA. mzach@mcw.edu
authors.forename: Michael
authors.initials: M
authors.lastname: Zacharisen
authors.forename: William
authors.initials: W
authors.lastname: Schoenwetter
mesh_terms: D000305:Adrenal Cortex Hormones / Q000494:pharmacology; D000542:Alveolitis, Extrinsic Allergic / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology*; D030161:Avian Proteins / Q000276:immunology; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008875:Middle Aged; D016273:Occupational Exposure
chemical_list: D000305:Adrenal Cortex Hormones; D030161:Avian Proteins