Chapter 4: Diseases of the Immune System
ICD-11 to ICD-10 crosswalk mappings for codes 4A00-4B4Z
74 ICD-11 codes in this chapter have ICD-10 crosswalk mappings from the WHO.
A new chapter in ICD-11 covering primary immunodeficiencies, autoimmune diseases, allergic and hypersensitivity conditions, and immune system disorders. This was separated from blood diseases to reflect modern understanding of immunology.
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4A00-4A0Z: Primary immunodeficiencies
21 codes with ICD-10 mappings
4A00.0ZFunctional neutrophil defects, unspecified1 mapping4A00.1ZDefects in the complement system, unspecified1 mapping4A01.00Hereditary agammaglobulinaemia with profoundly reduced or absent B cells1 mapping4A01.01Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells1 mapping4A01.02Specific antibody deficiency with normal immunoglobulin concentrations or normal number of B cells1 mapping4A01.03Transient hypogammaglobulinaemia of infancy1 mapping4A01.04Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells3 mappings4A01.05Immunodeficiencies with severe reduction in serum IgG or IgA with normal or elevated IgM and normal numbers of B-cells1 mapping4A01.0YOther specified immunodeficiencies with predominantly antibody defects7 mappings4A01.0ZImmunodeficiencies with predominantly antibody defects, unspecified2 mappings4A01.10Severe combined immunodeficiencies5 mappings4A01.11Major histocompatibility complex class I deficiency1 mapping4A01.12Major histocompatibility complex class II deficiency1 mapping4A01.1YOther specified combined immunodeficiencies1 mapping4A01.1ZCombined immunodeficiencies, unspecified3 mappings4A01.22Immune dysregulation syndromes presenting primarily with lymphoproliferation1 mapping4A01.23Primary haemophagocytic lymphohistiocytosis1 mapping4A01.30Immunodeficiency due to defects of the thymus1 mapping4A01.34Hyperimmunoglobulin E syndromes1 mapping4A01.ZPrimary immunodeficiencies due to disorders of adaptive immunity, unspecified1 mapping4A0ZPrimary immunodeficiencies, unspecified3 mappings
4A40-4A4Z: Nonorgan specific systemic autoimmune disorders
27 codes with ICD-10 mappings
4A40.0ZSystemic lupus erythematosus, unspecified4 mappings4A40.1Drug-induced lupus erythematosus1 mapping4A40.ZLupus erythematosus, unspecified2 mappings4A41.00Adult dermatomyositis1 mapping4A41.01Juvenile dermatomyositis1 mapping4A41.0ZDermatomyositis, unspecified2 mappings4A41.1ZPolymyositis, unspecified1 mapping4A41.20Inflammatory inclusion body myositis1 mapping4A41.2ZInclusion body myopathy, unspecified1 mapping4A41.ZIdiopathic inflammatory myopathy, unspecified2 mappings4A42.1Diffuse systemic sclerosis1 mapping4A42.2Limited systemic sclerosis1 mapping4A42.ZSystemic sclerosis, unspecified4 mappings4A43.4Diffuse eosinophilic fasciitis1 mapping4A43.ZUndifferentiated non-organ specific systemic autoimmune disease2 mappings4A44.1Aortic arch syndrome1 mapping4A44.2Giant cell arteritis1 mapping4A44.4Polyarteritis nodosa2 mappings4A44.5Mucocutaneous lymph node syndrome1 mapping4A44.8Thromboangiitis obliterans1 mapping4A44.92IgA vasculitis1 mapping4A44.A0Microscopic polyangiitis1 mapping4A44.A1Granulomatosis with polyangiitis1 mapping4A44.A2Eosinophilic granulomatosis with polyangiitis1 mapping4A44.YOther specified vasculitis2 mappings4A44.ZVasculitis, unspecified6 mappings4A4ZNonorgan specific systemic autoimmune disorders, unspecified6 mappings
4A60-4A6Z: Autoinflammatory disorders
1 code with ICD-10 mappings
4A80-4A8Z: Allergic or hypersensitivity conditions
8 codes with ICD-10 mappings
4A80.ZAllergic or hypersensitivity disorders involving the respiratory tract, unspecified1 mapping4A84.0Anaphylaxis due to allergic reaction to food1 mapping4A84.1Drug-induced anaphylaxis1 mapping4A84.YOther specified anaphylaxis1 mapping4A84.ZAnaphylaxis, unspecified1 mapping4A85.22Allergic contact dermatitis due to food allergen1 mapping4A85.2ZFood hypersensitivity, unspecified1 mapping4A8ZAllergic or hypersensitivity conditions of unspecified type1 mapping
4B00-4B0Z: Immune system disorders involving white cell lineages
4 codes with ICD-10 mappings
4B20-4B2Y: Certain disorders involving the immune system
9 codes with ICD-10 mappings
4B20.0Sarcoidosis of lung1 mapping4B20.1Sarcoidosis of lymph nodes1 mapping4B20.3Neurosarcoidosis1 mapping4B20.5Cutaneous sarcoidosis1 mapping4B20.ZSarcoidosis, unspecified3 mappings4B21Polyclonal hypergammaglobulinaemia1 mapping4B23Immune reconstitution inflammatory syndrome1 mapping4B24.ZGraft-versus-host disease, unspecified1 mapping4B2YOther specified disorders involving the immune system1 mapping