DB96.1 Primary biliary cholangitis International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01 Primary biliary cholangitis is characterised by progressive destruction and disappearance of the intralobular bile duct epithelial cells leading to cholestasis (high alkaline phosphatase and GGT {gamma glutamyl transferase}) and eventually liver cirrhosis and liver failure, generally associated with the presence of circulating antimitochondrial antibodies and an increase of serum IgM levels.
inclusions chronic nonsuppurative destructive cholangitis exclusions sections/codes in this section (DB96.1-DB96.1) postcoordination Add Stem and/or Extension codes to form a cluster code which adds detail to the condition.
Associated with - multiple selections are allowed [select] 4A00 Primary immunodeficiencies due to disorders of innate immunity – 4A00.0 Functional neutrophil defects – 4A00.1 Defects in the complement system – 4A00.2 Genetic susceptibility to particular pathogens – 4A00.3 Immunodeficiency with natural-killer cell deficiency – 4A00.Y Other specified primary immunodeficiencies due to disorders of innate immunity – 4A00.Z Primary immunodeficiencies due to disorders of innate immunity, unspecified –– 4A00.00 Neutrophil immunodeficiency syndrome –– 4A00.0Y Other specified functional neutrophil defects –– 4A00.0Z Functional neutrophil defects, unspecified –– 4A00.10 Immunodeficiency with an early component of complement deficiency –– 4A00.11 Immunodeficiency with a late component of complement deficiency –– 4A00.12 Immunodeficiency with factor B deficiency –– 4A00.13 Immunodeficiency with factor D anomaly –– 4A00.14 Hereditary angioedema –– 4A00.15 Acquired angioedema –– 4A00.1Y Other specified defects in the complement system –– 4A00.1Z Defects in the complement system, unspecified 4A01 Primary immunodeficiencies due to disorders of adaptive immunity – 4A01.0 Immunodeficiencies with predominantly antibody defects – 4A01.1 Combined immunodeficiencies – 4A01.2 Diseases of immune dysregulation – 4A01.3 Other well-defined immunodeficiency syndromes due to defects in adaptive immunity – 4A01.Z Primary immunodeficiencies due to disorders of adaptive immunity, unspecified –– 4A01.00 Hereditary agammaglobulinaemia with profoundly reduced or absent B cells –– 4A01.01 Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells –– 4A01.02 Specific antibody deficiency with normal immunoglobulin concentrations or normal number of B cells –– 4A01.03 Transient hypogammaglobulinaemia of infancy –– 4A01.04 Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells –– 4A01.05 Immunodeficiencies with severe reduction in serum IgG or IgA with normal or elevated IgM and normal numbers of B-cells –– 4A01.0Y Other specified immunodeficiencies with predominantly antibody defects –– 4A01.0Z Immunodeficiencies with predominantly antibody defects, unspecified –– 4A01.10 Severe combined immunodeficiencies –– 4A01.11 Major histocompatibility complex class I deficiency –– 4A01.12 Major histocompatibility complex class II deficiency –– 4A01.1Y Other specified combined immunodeficiencies –– 4A01.1Z Combined immunodeficiencies, unspecified –– 4A01.20 Immune dysregulation syndromes with hypopigmentation –– 4A01.21 Immune dysregulation syndromes presenting primarily with autoimmunity –– 4A01.22 Immune dysregulation syndromes presenting primarily with lymphoproliferation –– 4A01.23 Primary haemophagocytic lymphohistiocytosis –– 4A01.2Y Other specified diseases of immune dysregulation –– 4A01.2Z Diseases of immune dysregulation, unspecified –– 4A01.30 Immunodeficiency due to defects of the thymus –– 4A01.31 DNA repair defects other than combined T-cell or B-cell immunodeficiencies –– 4A01.32 Immuno-osseous dysplasia –– 4A01.33 Hepatic veno-occlusive disease - immunodeficiency syndrome –– 4A01.34 Hyperimmunoglobulin E syndromes 4A0Y Other specified primary immunodeficiencies 4A0Z Primary immunodeficiencies, unspecified 4A20 Acquired immunodeficiencies 4A20.0 Adult-onset immunodeficiency 4A20.1 Acquired immunodeficiency due to loss of immunoglobulin 4A20.Y Other specified acquired immunodeficiencies 4A20.Z Acquired immunodeficiencies, unspecified 4A40 Lupus erythematosus – 4A40.0 Systemic lupus erythematosus – 4A40.1 Drug-induced lupus erythematosus – 4A40.Y Other specified lupus erythematosus – 4A40.Z Lupus erythematosus, unspecified –– 4A40.00 Systemic lupus erythematosus with skin involvement –– 4A40.0Y Other specified systemic lupus erythematosus –– 4A40.0Z Systemic lupus erythematosus, unspecified 4A41 Idiopathic inflammatory myopathy – 4A41.0 Dermatomyositis – 4A41.1 Polymyositis – 4A41.2 Inclusion body myopathy – 4A41.Y Other specified idiopathic inflammatory myopathy – 4A41.Z Idiopathic inflammatory myopathy, unspecified –– 4A41.00 Adult dermatomyositis –– 4A41.01 Juvenile dermatomyositis –– 4A41.0Z Dermatomyositis, unspecified –– 4A41.10 Juvenile polymyositis –– 4A41.11 Paraneoplastic polymyositis –– 4A41.1Y Other specified polymyositis –– 4A41.1Z Polymyositis, unspecified –– 4A41.20 Inflammatory inclusion body myositis –– 4A41.21 Noninflammatory inclusion body myopathy –– 4A41.2Z Inclusion body myopathy, unspecified 4A42 Systemic sclerosis – 4A42.0 Paediatric onset systemic sclerosis – 4A42.1 Diffuse systemic sclerosis – 4A42.2 Limited systemic sclerosis – 4A42.Z Systemic sclerosis, unspecified 4A43 Overlap or undifferentiated nonorgan specific systemic autoimmune disease – 4A43.0 IgG4 related disease – 4A43.1 Mikulicz disease – 4A43.2 Sjögren syndrome – 4A43.3 Mixed connective tissue disease – 4A43.4 Diffuse eosinophilic fasciitis – 4A43.Y Other specified overlap non-organ specific systemic autoimmune disease – 4A43.Z Undifferentiated non-organ specific systemic autoimmune disease –– 4A43.20 Primary Sjögren syndrome –– 4A43.21 Secondary Sjögren syndrome –– 4A43.22 Paediatric onset Sjögren syndrome –– 4A43.2Y Other specified sjögren syndrome –– 4A43.2Z Sjögren syndrome, unspecified 4A44 Vasculitis – 4A44.0 Rhizomelic pseudopolyarthritis – 4A44.1 Aortic arch syndrome – 4A44.2 Giant cell arteritis – 4A44.3 Single organ vasculitis – 4A44.4 Polyarteritis nodosa – 4A44.5 Mucocutaneous lymph node syndrome – 4A44.6 Sneddon syndrome – 4A44.7 Primary angiitis of the central nervous system – 4A44.8 Thromboangiitis obliterans – 4A44.9 Immune complex small vessel vasculitis – 4A44.A Antineutrophil cytoplasmic antibody-associated vasculitis – 4A44.B Leukocytoclastic vasculitis – 4A44.Y Other specified vasculitis – 4A44.Z Vasculitis, unspecified –– 4A44.90 Cryoglobulinaemic vasculitis –– 4A44.91 Hypocomplementaemic urticarial vasculitis –– 4A44.92 IgA vasculitis –– 4A44.9Y Other specified immune complex small vessel vasculitis –– 4A44.9Z Immune complex small vessel vasculitis, unspecified –– 4A44.A0 Microscopic polyangiitis –– 4A44.A1 Granulomatosis with polyangiitis –– 4A44.A2 Eosinophilic granulomatosis with polyangiitis –– 4A44.AY Other specified antineutrophil cytoplasmic antibody-associated vasculitis –– 4A44.AZ Antineutrophil cytoplasmic antibody-associated vasculitis, unspecified –– 4A44.B0 Cutaneous leukocytoclastic vasculitis –– 4A44.BY Other specified leukocytoclastic vasculitis –– 4A44.BZ Leukocytoclastic vasculitis, unspecified 4A45 Antiphospholipid syndrome – 4A45.0 Primary antiphospholipid syndrome – 4A45.1 Secondary antiphospholipid syndrome – 4A45.2 Antiphospholipid syndrome in pregnancy – 4A45.3 Lupus anticoagulant-hypoprothrombinaemia syndrome – 4A45.Z Antiphospholipid syndrome, unspecified 4A4Y Other specified nonorgan specific systemic autoimmune disorders 4A4Z Nonorgan specific systemic autoimmune disorders, unspecified 4A60 Monogenic autoinflammatory syndromes – 4A60.0 Familial Mediterranean fever – 4A60.1 Cryopyrin-associated periodic syndromes – 4A60.2 Tumour necrosis factor receptor 1 associated periodic syndrome – 4A60.Y Other specified monogenic autoinflammatory syndromes – 4A60.Z Autoimflammatory syndrome, unspecified 4A61 SAPHO syndrome 4A62 Behçet disease 4A6Y Other specified autoinflammatory disorders 4A6Z Autoinflammatory disorders, unspecified 4A80 Allergic or hypersensitivity disorders involving the respiratory tract – 4A80.0 Drug-induced bronchospasm – 4A80.1 Bronchospasm provoked by allergy to food substance – 4A80.Y Other specified allergic or hypersensitivity disorders involving the respiratory tract – 4A80.Z Allergic or hypersensitivity disorders involving the respiratory tract, unspecified 4A81 Allergic or hypersensitivity disorders involving the eye 4A82 Allergic or hypersensitivity disorders involving skin or mucous membranes 4A83 Allergic or hypersensitivity disorders involving the gastrointestinal tract – 4A83.0 Food-induced eosinophilic gastroenteritis – 4A83.1 Food-induced eosinophilic oesophagitis – 4A83.Y Other specified allergic or hypersensitivity disorders involving the gastrointestinal tract – 4A83.Z Allergic or hypersensitivity disorders involving the gastrointestinal tract, unspecified 4A84 Anaphylaxis – 4A84.0 Anaphylaxis due to allergic reaction to food – 4A84.1 Drug-induced anaphylaxis – 4A84.2 Anaphylaxis due to insect venom – 4A84.3 Anaphylaxis provoked by physical factors – 4A84.4 Anaphylaxis due to inhaled allergens – 4A84.5 Anaphylaxis due to contact with allergens – 4A84.6 Anaphylaxis secondary to mast cell disorder – 4A84.Y Other specified anaphylaxis – 4A84.Z Anaphylaxis, unspecified –– 4A84.30 Exercise-induced anaphylaxis –– 4A84.31 Cold-induced anaphylaxis –– 4A84.3Y Anaphylaxis provoked by other specified physical factors –– 4A84.3Z Anaphylaxis provoked by unspecified physical factors 4A85 Complex allergic or hypersensitivity conditions – 4A85.0 Drug or pharmacological agents hypersensitivity – 4A85.1 Hypersensitivity to herbal and alternative medical therapies – 4A85.2 Food hypersensitivity – 4A85.3 Allergic or hypersensitivity reactions to arthropods – 4A85.Y Other specified complex allergic or hypersensitivity conditions – 4A85.Z Complex allergic or hypersensitivity conditions, unspecified –– 4A85.00 Drug-induced liver hypersensitivity disease –– 4A85.01 Drug-induced kidney hypersensitivity –– 4A85.02 Drug-induced cytopenia –– 4A85.03 Drug-induced vasculitis –– 4A85.04 Multiple drug hypersensitivity syndrome –– 4A85.0Y Drug hypersensitivity of other specified type –– 4A85.0Z Drug hypersensitivity of unspecified type –– 4A85.20 Food-induced gastrointestinal hypersensitivity –– 4A85.21 Food-induced urticaria or angioedema –– 4A85.22 Allergic contact dermatitis due to food allergen –– 4A85.2Y Other specified food hypersensitivity –– 4A85.2Z Food hypersensitivity, unspecified –– 4A85.30 Systemic allergic reaction due to Hymenoptera venom –– 4A85.31 Cutaneous allergic or hypersensitivity reactions to Hymenoptera venom –– 4A85.32 Cutaneous allergic or hypersensitivity reactions to arthropods 4A8Y Allergic or hypersensitivity conditions of other specified type 4A8Z Allergic or hypersensitivity conditions of unspecified type 4B00 Disorders of neutrophil number – 4B00.0 Neutropaenia – 4B00.1 Neutrophilia – 4B00.Y Other specified disorders of neutrophil number –– 4B00.00 Constitutional neutropaenia –– 4B00.01 Acquired neutropaenia –– 4B00.0Z Neutropaenia, unspecified –– 4B00.10 Constitutional neutrophilia –– 4B00.11 Acquired neutrophilia –– 4B00.1Z Neutrophilia, unspecified 4B01 Disorders of neutrophil function – 4B01.0 Constitutional disorders of neutrophil function – 4B01.1 Acquired disorders of neutrophil function – 4B01.Z Disorders of neutrophil function, unspecified –– 4B01.00 Disorders of neutrophil adhesion –– 4B01.01 Disorders of neutrophil chemotaxis –– 4B01.02 Disorders of neutrophil granule formation or release –– 4B01.03 Disorders of neutrophil oxidative metabolism –– 4B01.0Y Other specified constitutional disorders of neutrophil function –– 4B01.0Z Constitutional disorders of neutrophil function, unspecified 4B02 Eosinopenia – 4B02.0 Constitutional decrease in eosinophil number – 4B02.1 Acquired decrease in eosinophil number – 4B02.Z Eosinopenia, unspecified 4B03 Eosinophilia – 4B03.0 Constitutional eosinophilia – 4B03.1 Acquired eosinophilia – 4B03.Z Eosinophilia, unspecified 4B04 Disorders with decreased monocyte counts 4B05 Disorders with increased monocyte counts 4B06 Acquired lymphopenia 4B07 Acquired lymphocytosis 4B0Y Other specified immune system disorders involving white cell lineages 4B0Z Immune system disorders involving white cell lineages, unspecified 4B20 Sarcoidosis – 4B20.0 Sarcoidosis of lung – 4B20.1 Sarcoidosis of lymph nodes – 4B20.2 Sarcoidosis of the digestive system – 4B20.3 Neurosarcoidosis – 4B20.4 Ocular sarcoidosis – 4B20.5 Cutaneous sarcoidosis – 4B20.Y Other specified sarcoidosis – 4B20.Z Sarcoidosis, unspecified 4B21 Polyclonal hypergammaglobulinaemia 4B22 Cryoglobulinaemia 4B23 Immune reconstitution inflammatory syndrome 4B24 Graft-versus-host disease – 4B24.0 Acute graft-versus-host disease – 4B24.1 Chronic graft-versus-host disease – 4B24.Y Other specified graft-versus-host disease – 4B24.Z Graft-versus-host disease, unspecified 4B2Y Other specified disorders involving the immune system 4B40 Diseases of thymus 4B40.0 Persistent hyperplasia of thymus 4B40.1 Abscess of thymus 4B40.2 Good syndrome 4B40.Y Other specified diseases of thymus 4B40.Z Diseases of thymus, unspecified 4B4Y Other specified diseases of the immune system 4B4Z Diseases of the immune system, unspecified – DB93.1 Hepatic cirrhosis – ME24 Clinical manifestations of the digestive system –– ME24.0 Digestive system abscess –– ME24.1 Digestive system fistula –– ME24.2 Digestive system obstruction –– ME24.3 Digestive system perforation –– ME24.4 Digestive system stenosis –– ME24.5 Digestive system ulcer –– ME24.6 Digestive system dilatation –– ME24.7 Digestive system incarceration –– ME24.8 Digestive system strangulation or gangrene –– ME24.9 Gastrointestinal bleeding –– ME24.A Other digestive system haemorrhage, not elsewhere classified –– ME24.Y Other specified clinical manifestations of the digestive system ––– ME24.30 Perforation of small intestine ––– ME24.31 Perforation of large intestine ––– ME24.35 Perforation of gallbladder or bile ducts ––– ME24.3Y Digestive system perforation of other specified site ––– ME24.3Z Digestive system perforation of unspecified site ––– ME24.90 Acute gastrointestinal bleeding, not elsewhere classified ––– ME24.91 Chronic gastrointestinal bleeding, not elsewhere classified ––– ME24.9Z Gastrointestinal bleeding, unspecified ––– ME24.A0 Obscure gastrointestinal bleeding ––– ME24.A1 Haemorrhage of anus and rectum ––– ME24.A2 Oesophageal haemorrhage ––– ME24.A3 Haematochezia ––– ME24.A4 Melaena ––– ME24.A5 Haematemesis ––– ME24.A6 Positive occult blood in stool Course - only one may be selected [select] XT5R Acute XT8W Chronic
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