Localised forms of pulmonary amyloidosis

Vnitr Lek. 2021 Winter;67(6):357-364.

Abstract

Amyloidosis is a rare disorder caused by amyloid deposits in various organs and tissues resulting in vital organ dysfunction, eventually death. There are two forms of amyloidosis - systemic, characterized by multiple organs affected, and localized (focal). Localized forms of amyloidosis usually affect urinary bladder, skin and lungs. Pulmonary amyloidosis may be localized or systemic such as diffuse alveolo-septal pulmonary amyloidosis which usually accompanies systemic AL amyloidosis. Other two forms of pulmonary amyloidosis are tracheobronchial and nodular. All three forms are usually detected by accident when patients undergo chest examination for different reasons as most cases of pulmonary amyloidosis are asymptomatic. The prognosis of localized amyloidosis is good with 5-year overall survival being 90,6 %. In our case report we present three patients diagnosed with localized pulmonary amyloidosis at our center. In all cases the diagnoses were made following the resection of affected lung segments with no further treatment needed.

Keywords: Amyloidosis; alveolo-septal amyloidosis; amyloidosis; localized amyloidosis; nodular pulmonary amyloidosis; tracheobronchial amyloidosis.

Publication types

  • Case Reports

MeSH terms

  • Amyloidosis* / diagnosis
  • Amyloidosis* / therapy
  • Humans
  • Immunoglobulin Light-chain Amyloidosis* / complications
  • Immunoglobulin Light-chain Amyloidosis* / diagnosis
  • Lung Diseases* / diagnosis
  • Prognosis