Disorder of immune function	Hereditary disorder by system
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Hereditary disorder of immune system (disorder)
	Hereditary disorder of immune system
	hereditaire aandoening van immuunsysteem
	erfelijke ziekte van afweersysteem

Id	363138005
Status	Defined

Finding site

Body system structure

Pathological process

Abnormal immune process

ICD-10 complex map reference set

Target	D89.9
Rule	TRUE
Advice	ALWAYS D89.9
Correlation	SNOMED CT source code to target map code correlation not specified

Absent thumb with short stature and immunodeficiency syndrome

Agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis syndrome

Alopecia, psychomotor epilepsy, periodontal pyorrhea, intellectual disability syndrome

Ataxia-telangiectasia syndrome

Autoimmune enteropathy and endocrinopathy with susceptibility to chronic infection syndrome

Autoimmune interstitial lung disease, arthritis syndrome

Autoimmune lymphoproliferative syndrome2

Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation

Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis

CEBPE-associated autoinflammation, immunodeficiency, neutrophil dysfunction syndrome

Combined immunodeficiency due to OX40 deficiency

Combined immunodeficiency with faciooculoskeletal anomalies syndrome

Common variable immunodeficiency with autoantibodies to B- or T-cells

Common variable immunodeficiency with predominant abnormalities of B-cell numbers and functions

Common variable immunodeficiency with predominant immunoregulatory T-cell disorders

Complement component 3 deficiency

Complement component deficiency

Congenital sideroblastic anemia, B-cell immunodeficiency, periodic fever, developmental delay syndrome

Constitutional mismatch repair deficiency syndrome

Cystic fibrosis with allergic bronchopulmonary aspergillosis

Deficiency in anterior pituitary function, variable immunodeficiency syndrome

Deficiency of interleukin 36 receptor antagonist

Facial dysmorphism, immunodeficiency, livedo, short stature syndrome

Familial amyloid nephropathy with urticaria AND deafness

Familial C3B inhibitor deficiency syndrome

Familial chilblain lupus erythematosus

Familial cold urticaria1

Genetically determined myasthenia6

Granulomatous inflammatory arthritis, dermatitis and uveitis, familial

Hennekam syndrome

Hereditary C1 esterase inhibitor deficiency - deficient factor

Hereditary C1 esterase inhibitor deficiency - dysfunctional factor

Hereditary hypogammaglobulinemia1

Hereditary white blood cell disorder25

Immune dysregulation, inflammatory bowel disease, arthritis, recurrent infection syndrome

Immunodeficiency with factor I anomaly

Infantile inflammatory bowel disease with neurological involvement

Isolated agammaglobulinemia1

Lichtenstein syndrome

Majeed syndrome

Microcephaly, normal intelligence and immunodeficiency

Multiple sclerosis, ichthyosis, factor VIII deficiency syndrome

Nakajo-Nishimura syndrome

Neonatal inflammatory skin and bowel disease

NLRP12-associated hereditary periodic fever syndrome

Osteopetrosis hypogammaglobulinemia syndrome

Polyglandular autoimmune syndrome, type 1

Primary CD59 deficiency

Primary immunodeficiency with natural killer cell deficiency and adrenal insufficiency

Pyogenic arthritis, pyoderma gangrenosum, acne syndrome

Recurrent Neisseria infection due to factor D deficiency

Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations

Roifman syndrome

Schimke immuno-osseous dysplasia

Severe combined immunodeficiency disease18

Severe dermatitis, multiple allergies, metabolic wasting syndrome

Sterile multifocal osteomyelitis with periostitis and pustulosis

STING-associated vasculopathy with onset in infancy

Vasculitis due to adenosine deaminase 2 deficiency

Vici syndrome

Warts, immunodeficiency, lymphedema, anogenital dysplasia syndrome

Wiskott-Aldrich syndrome1

X-linked agammaglobulinemia with growth hormone deficiency

X-linked immune dysregulation, polyendocrinopathy, enteropathy syndrome

X-linked immunoneurologic disorder

X-linked intellectual disability with seizure and psoriasis syndrome

X-linked lymphoproliferative syndrome4