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AApoAIV amyloidosis

Disease definition

A rare nonhereditary systemic amyloidosis characterized by slowly progressive renal dysfunction, increased serum creatinine, mostly normal urine analysis with no significant proteinuria and associated heart disease. Cardiac involvement presents as hypertrophic obstructive cardiomyopathy, left ventricular outflow tract obstruction, coronary artery disease and conduction system abnormalities. Histology reveals medullar amyloid deposits, renal tubular atrophy, interstitial fibrosis, and glomerular sclerosis.

ORPHA:439232

Classification level: Disorder
  • Synonym(s):
    • Apolipoprotein A-IV amyloidosis
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: E85.8
  • ICD-11: 5D00.2Y
  • OMIM: -
  • UMLS: C5568805
  • MeSH: -
  • GARD: -
  • MedDRA: -

  A summary on this disease is available in Italiano (2018) Español (2023) Français (2023) Nederlands (2023)

Detailed information

General public

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.