5D00.1

ICD-11 MMS 2026-01

AA amyloidosis

Description

AA (Amyloid A) amyloidosis is due to the deposition of the acute phase reactant serum amyloid A protein (SAA) in glomeruli where they are seen as Congo red binding fibrils which immunostain specifically for SAA. Chronic inflammation is responsible. Other organs are also involved in this systemic disease.

Index terms

  • AA amyloidosis
  • AA - [Amyloid A]
  • Secondary amyloidosis
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  • Secondary systemic amyloidosis
  • reactive systemic amyloidosis
  • Cardiac secondary systemic amyloidosis
  • Cerebral secondary systemic amyloidosis angiopathy
  • Glomerular disorders in secondary systemic amyloidosis

Exclusions from above levels

  • 6D80 – Dementia due to Alzheimer disease
  • 2B31.2 – histiocytosis X (chronic)
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
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  • 5A71.01 – Congenital adrenal hyperplasia
  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5D00.0 – AL amyloidosis
  • 5D00.2 – Hereditary amyloidosis
  • 5D00.3 – Dialysis-associated amyloidosis
  • 5D00.Y – Other specified amyloidosis
  • 5D00.Z – Amyloidosis, unspecified

Foundation entity

AA amyloidosis – http://id.who.int/icd/entity/570181034

Allowed postcoordination axes

Part of: 5D00: Amyloidosis › 05: Endocrine, nutritional or metabolic diseases