Pulmonary amyloidosis in familial mediterranean fever


Sahan C., Cengiz K.

ACTA CLINICA BELGICA, vol.61, no.3, pp.147-151, 2006 (SCI-Expanded) identifier

  • Publication Type: Article / Article
  • Volume: 61 Issue: 3
  • Publication Date: 2006
  • Journal Name: ACTA CLINICA BELGICA
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.147-151
  • Keywords: lung, kidney, amyloidosis, familial mediterranean fever, CT APPEARANCE, MUTATIONS, FEATURES, DISEASE, GENE
  • Ondokuz Mayıs University Affiliated: No

Abstract

Familial Mediterranean Fever (FMF) is a hereditary periodic fever syndrome expressed by acute episodes of fever and painful manifestations. The gravest consequence of FMF is kidney involvement by secondary amyloidosis of AA type, which gradually leads to nephrotic syndrome and uremia. Nephropathic amyloidosis of the AA type, which complicates FMF in most untreated patients, may progress to effect other organs, including the lungs. This kind of organ involvement rarely produces noticeable symptoms and is associated with symptomatic involvement of other organs while remaining subclinical in itself. In this report, one case who had nephropathic and pulmonary amyloidosis of the secondary amyloidosis of AA type, wich complicates the FMF was presented and the pulmonary manifestations of FMF were reviewed.