Certain Disorders Involving The Immune System ICD 11

Definitions of Certain Disorders Involving The Immune System: Disorders in which disturbed immune regulation plays an important part but which cannot be more precisely located elsewhere in the classification.


  • Failure or rejection of transplanted organs or tissues (NE84)
  • Monoclonal gammopathy of undetermined significance (2A83.0)

Coded Elsewhere:

  • Hereditary angioedema (4A00.14)


Definitions of Sarcoidosis: Sarcoidosis is a multisystem disorder of unknown cause characterised by the formation of immune granulomas in involved organs. The lung and the lymphatic system are predominantly affected, but virtually every organ may be involved. Other severe manifestations result from cardiac, neurological, ocular, kidney or laryngeal localizations.

ICD 11 Code For Sarcoidosis

  4B20  Sarcoidosis

4B20.0 Sarcoidosis of lung

4B20.1 Sarcoidosis of lymph nodes

Definitions of Sarcoidosis of lymph nodes: Lymphadenopathy is very common in sarcoidosis. Intrathoracic nodes are enlarged in 75 to 90% of all patients; usually this involves the hilar nodes, but the paratracheal nodes are commonly involved. Peripheral lymphadenopathy is very common, particularly involving the cervical (the most common head and neck manifestation of the disease), axillary, epitrochlear, and inguinal nodes.

4B20.2 Sarcoidosis of the digestive system

Definitions of Sarcoidosis of the digestive system: This is a syndrome involving abnormal collections of chronic inflammatory cells (granulomas) that can form as nodules in the digestive system.

Coded Elsewhere:

  • Gastritis due to sarcoidosis (DA42.Y)
  • Oesophagitis due to sarcoidosis (DA24.Y)

4B20.3 Neurosarcoidosis

Definitions of Neurosarcoidosis: This refers to sarcoidosis, a condition of unknown cause featuring granulomas in various tissues, involving the central nervous system (brain and spinal cord). It can have many manifestations, but abnormalities of the cranial nerves (a group of twelve nerves supplying the head and neck area) are the most common.

4B20.4 Ocular sarcoidosis

Definitions of Ocular sarcoidosis: This is a syndrome involving abnormal collections of chronic inflammatory cells (granulomas) that can form as nodules in multiple organs.

Coded Elsewhere:

  • Uveoparotid fever (4B20.Y)

4B20.5 Cutaneous sarcoidosis

4B20.Y Other specified sarcoidosis

4B20.Z Sarcoidosis unspecified

Polyclonal hypergammaglobulinaemia

ICD 11 Code For Polyclonal hypergammaglobulinaemia

  4B21  Polyclonal hypergammaglobulinaemia


ICD 11 Code For Cryoglobulinaemia

  4B22  Cryoglobulinaemia

Coded Elsewhere:

  • Cryoglobulinaemic vasculitis (4A44.90)
  • Cutaneous microvascular disturbances due to monoclonal cryoglobulins (EF5Y)

Immune reconstitution inflammatory syndrome

Definitions of Immune reconstitution inflammatory syndrome: This is a condition seen in some cases of AIDS or immunosuppression, in which the immune system begins to recover, but then responds to a previously acquired opportunistic infection with an overwhelming inflammatory response that paradoxically makes the symptoms of infection worse.

ICD 11 Code For Immune reconstitution inflammatory syndrome

  4B23  Immune reconstitution inflammatory syndrome

Graft-versus-host disease

Definitions of Graft-versus-host disease: Graft-versus-host disease (GVHD) occurs when lymphoid cells from an immunocompetent donor are introduced into a histo-incompatible recipient incapable of rejecting them. This usually occurs as a result of haematopoietic stem cell transplantation. The main targets attacked by the donor lymphocytes are the recipient’s skin, gastrointestinal tract and liver. Acute GVHD, normally occurring within the first 100 days following transplantation, has a high mortality. The acute phase may be followed by chronic GVHD, which can also arise de novo. This usually presents as a lichenoid rash but can develop into a severe fibrosing disease affecting skin, lungs and liver.

ICD 11 Code For Graft-versus-host disease

  4B24  Graft-versus-host disease

4B24.0 Acute graft-versus-host disease

Definitions of Acute graft-versus-host disease: Graft-versus-host disease presenting normally within the first 100 days of engraftment. It presents most commonly with a maculopapular rash accompanied by fever. The prognosis correlates with the extent of skin involvement, which may progress to widespread epidermal necrolysis, and the severity of gastrointestinal and liver involvement which may manifest as diarrhoea and jaundice respectively. There is a high mortality in severe acute graft-versus-host disease.

4B24.1 Chronic graft-versus-host disease

Definitions of Chronic graft-versus-host disease: Chronic graft-versus-host disease (GVHD) presents more than 100 days after engraftment of immunocompetent donor lymphoid cells. It has specific clinical features by which it can be distinguished from acute GVHD. It may arise de novo but frequently follows acute GVHD. Less commonly, it occurs concurrently with acute GVHD. The earlier stages of chronic GVHD are characterised by a widespread lichenoid rash, poikiloderma and involvement of nails and oral mucous membranes. If the disease remains active, progressive sclerosis of the skin and deeper tissues may result in joint contractures, fibrosis of internal organs and severe malabsorption.

4B24.Y Other specified graft-versus-host disease

4B24.Z Graft-versus-host disease unspecified

Other specified disorders involving the immune system

ICD 11 Code For Other specified disorders involving the immune system

  4B2Y  Other specified disorders involving the immune system

Diseases of thymus

ICD 11 Code For Diseases of thymus

  4B40  Diseases of thymus


  • thymic aplasia or hypoplasia with immunodeficiency (LD44.N0)
  • Myasthenia gravis (8C60)

Coded Elsewhere:

  • Thymic tumours

4B40.0 Persistent hyperplasia of thymus

Definitions of Persistent hyperplasia of thymus: This refers to a persistent enlargement (“hyperplasia”) of the thymus.

4B40.1 Abscess of thymus

4B40.2 Good syndrome

Definitions of Good syndrome: This is a condition that occurs in adults in whom hypogammaglobulinemia, deficient cell-mediated immunity, and benign thymoma may develop almost simultaneously.

4B40.Y Other specified diseases of thymus

4B40.Z Diseases of thymus unspecified

Other specified diseases of the immune system

ICD 11 Code For Other specified diseases of the immune system

  4B4Y  Other specified diseases of the immune system

Diseases of the immune system unspecified

ICD 11 Code For Diseases of the immune system unspecified

  4B4Z  Diseases of the immune system unspecified

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