title: Transbronchial biopsies safely diagnose amyloid lung disease.
abstract: BACKGROUND
  Autopsy identifies lung involvement in 58-92% of patients with the most prevalent forms of systemic amyloidoses. In the absence of lung biopsies, amyloid lung disease often goes unrecognized. Report of a death following transbronchial biopsies in a patient with systemic amyloidosis cautioned against the procedure in this patient cohort. We reviewed our experience with transbronchial biopsies in patients with amyloidosis to determine the safety and utility of bronchoscopic lung biopsies.
  
  
  METHODS
  We identified patients referred to the Amyloidosis Center at Boston Medical Center with lung amyloidosis diagnosed by transbronchial lung biopsies (TBBX). Amyloid typing was determined by immunohistochemistry or mass spectrometry. Standard end organ assessments, including pulmonary function test (PFT) and chest tomography (CT) imaging, and extra-thoracic biopsies established the extent of disease.
  
  
  RESULTS
  Twenty-five (21.7%) of 115 patients with lung amyloidosis were diagnosed by TBBX. PFT classified 33.3% with restrictive physiology, 28.6% with obstructive disease, and 9.5% mixed physiology; 9.5% exhibited isolated diffusion defects while 19% had normal pulmonary testing. Two view chest or CT imaging identified focal opacities in 52% of cases and diffuse interstitial disease in 48%. Amyloid type and disease extent included 68% systemic AL disease, 16% localized (lung limited) AL disease, 12% ATTR disease, and 4% AA amyloidosis. Fluoroscopy was not used during biopsy. No procedure complications were reported.
  
  
  CONCLUSIONS
  Our case series of 25 patients supports the use of bronchoscopic transbronchial biopsies for diagnosis of parenchymal lung amyloidosis. Normal PFTs do not rule out the histologic presence of amyloid lung disease.
authors.affiliation: a Pulmonary Center, Boston Medical Center , Boston , MA , USA.|b Department of Medicine , Boston Medical Center , Boston , MA , USA.
authors.forename: Praveen
authors.initials: P
authors.lastname: Govender
authors.affiliation: c Massachusetts General Hospital , Boston , MA , USA.
authors.forename: Colleen M
authors.initials: CM
authors.lastname: Keyes
authors.affiliation: d Brigham and Women's Hospital , Boston , MA , USA.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Hankinson
authors.affiliation: e Amyloidosis Center, Boston Medical Center , Boston , MA , USA.|f Department of Pathology , Boston Medical Center , Boston , MA , USA.
authors.forename: Carl J
authors.initials: CJ
authors.lastname: O'Hara
authors.affiliation: b Department of Medicine , Boston Medical Center , Boston , MA , USA.|e Amyloidosis Center, Boston Medical Center , Boston , MA , USA.
authors.forename: Vaishali
authors.initials: V
authors.lastname: Sanchorawala
authors.affiliation: a Pulmonary Center, Boston Medical Center , Boston , MA , USA.|b Department of Medicine , Boston Medical Center , Boston , MA , USA.|e Amyloidosis Center, Boston Medical Center , Boston , MA , USA.
authors.forename: John L
authors.initials: JL
authors.lastname: Berk
mesh_terms: D000328:Adult; D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000378:metabolism*; D001706:Biopsy / Q000379:methods*; D005260:Female; D006801:Humans; D008168:Lung / Q000378:metabolism* / Q000473:pathology; D008171:Lung Diseases / Q000378:metabolism*; D017563:Lung Diseases, Interstitial / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests
chemical_list: D000682:Amyloid
keywords: Lung biopsy; amyloidosis; interstitial lung disease