Localized interstitial pulmonary amyloid: a case report and review of the literature

Curr Opin Pulm Med. 2009 Sep;15(5):517-20. doi: 10.1097/MCP.0b013e32832d03d8.

Abstract

Purpose of review: Diffuse interstitial pulmonary amyloidosis is a fatal disorder that is rare and often undiagnosed. There are many documented cases of diffuse interstitial amyloid occurring with systemic amyloid light-chain amyloid. We present an extraordinary case of localized interstitial amyloidosis, making our case the eleventh documented in the literature.

Recent findings: We review the subtypes of tracheobronchial-pulmonary amyloidosis and their clinical presentations. There is also a thorough investigation of the literature published on localized diffuse interstitial pulmonary amyloid, and we provided information on the recent advances in treatment and diagnosis of amyloid.

Summary: Although localized diffuse interstitial amyloid is a rare finding, it is often not placed in the differential diagnosis, leading to the diagnosis being made at autopsy. With the use of new techniques and fiberoptic bronchoscopy an early diagnosis can be made. More research needs to be dedicated to the advancement of treatment for this fatal disease.

Publication types

  • Case Reports

MeSH terms

  • Amyloidosis / diagnosis*
  • Amyloidosis / drug therapy
  • Biopsy
  • Bronchoscopy
  • Diagnosis, Differential
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / drug therapy
  • Middle Aged
  • Radiography, Thoracic

Substances

  • Glucocorticoids