Other Metabolic Disorders – Definitions & ICD 11 Codes


  • histiocytosis X (chronic) (2B31.2)

Coded Elsewhere:

  • Tophaceous gout (FA25.20)


Definition of Amyloidosis: Amyloidosis is a vast group of diseases defined by the presence of insoluble protein deposits in tissues. Its diagnosis is based on histological findings. Amyloidoses are classified according to clinical signs and biochemical type of amyloid protein involved. Most amyloidoses are multisystemic, ‘generalised’ or ‘diffuse’. There are a few forms of localised amylosis. The most frequent forms are AL amyloidosis (immunoglobulins), AA (inflammatory), and ATTR (transthyretin accumulation).

ICD 11 Code For Amyloidosis

  5D00  Amyloidosis


Coded Elsewhere:

5D00.0 AL amyloidosis

Definition of AL amyloidosis: AL amyloid is due to the deposition of immunoglobulin light chains in glomeruli where they are seen as Congo red binding fibrils and immuno-stain specifically for kappa or lambda light chains. By light microscopy there is amorphous hyaline material in the mesangium and capillary walls. A light chain producing plasma cell or B-cell dysplasia is responsible. Other organs are also involved in this systemic disease.

Coded Elsewhere:

  • Isolated cerebral amyloid angiopathy (8B22.3)

5D00.1 AA amyloidosis

Definition of AA amyloidosis: AA amyloid 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 immuno- stain specifically for SAA. Chronic inflammation is responsible. Other organs are also involved in this systemic disease.

5D00.2 Hereditary amyloidosis

Definition of Hereditary amyloidosis: Hereditary amyloidosis (familial amyloidosis) is an inherited disorder that often affects the liver, nerves, heart and kidneys. Many different types of gene abnormalities present at birth are associated with an increased risk of amyloid disease. The type and location of an amyloid gene abnormality can affect the risk of certain complications, the age at which symptoms first appear, and the way the disease progresses over time.

5D00.20 Hereditary ATTR amyloidosis

5D00.21 Non-neuropathic heredofamilial amyloidosis

Definition of Non-neuropathic heredofamilial amyloidosis: This is an amyloidosis (the formation of insoluble proteins, or amyloids) of inherited origin that does not affect the peripheral nerves. The most common sites of deposits are associated with the kidney and heart.

Coded Elsewhere:

5D00.2Y Other specified hereditary amyloidosis

5D00.2Z Hereditary amyloidosis unspecified

5D00.3 Dialysis-associated amyloidosis

Definition of Dialysis-associated amyloidosis: Dialysis-related amyloidosis develops when proteins in blood are deposited in joints and tendons — causing pain, stiffness and fluid in the joints, as well as carpal tunnel syndrome. This type generally affects people on long-term dialysis.

5D00.Y Other specified amyloidosis

5D00.Z Amyloidosis unspecified

Definition of Amyloidosis unspecified: This is a group of metabolic complications that can occur after treatment of cancer, usually lymphomas and leukaemias, and sometimes even without treatment. These complications are caused by the breakdown products of dying cancer cells and include hyperkalaemia, hyperphosphataemia, hyperuricaemia and hyperuricosuria, hypocalcaemia, and consequent acute uric acid nephropathy and acute renal failure.

Tumour lysis syndrome

ICD 11 Code For Tumour lysis syndrome

  5D01  Tumour lysis syndrome

Coding Note:

  • Code also the causing condition

Other specified metabolic disorders

ICD 11 Code For Other specified metabolic disorders

  5D0Y  Other specified metabolic disorders

Metabolic disorders unspecified

ICD 11 Code For Metabolic disorders unspecified

  5D2Z  Metabolic disorders unspecified

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