AutoICD API

D84.1

Billable

Defects in the complement system

Defects in the complement system

Status

Billable / Specific

Block

D80-D89

Parent Code

D84

ICD-11 Mapping

1 equivalent

Coding Notes

Inclusion Terms

Alternative clinical terms for this condition

  • C1 esterase inhibitor [C1-INH] deficiency

Includes

Conditions included under this code

  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(3)
D84.0

Lymphocyte function antigen-1 [LFA-1] defect

BillableD84.8

Other specified immunodeficiencies

D84.9

Immunodeficiency, unspecified

Billable
ICD-11 Equivalents(1)

ICD-11 Equivalents

View full mapping

Corresponding ICD-11 codes from the WHO crosswalk mapping

4A00.1ZDefects in the complement system, unspecifiedequivalentPrimary
Also Known As / Clinical Terms(294)

SNOMED CT

UMLS

Clinical Terms

  • Angioedema due to disorder of kinin metabolism
  • Hereditary angioedema with C1 esterase inhibitor deficiency
  • complement deficiency
  • Protein-losing enteropathy
  • Complement 1q beta chain deficiency
  • Complement 6 deficiency
  • Carboxypeptidase N deficiency
  • HAE - Hereditary angioedema
  • Complement 7 deficiency
  • Atypical hemolytic uremic syndrome
  • Classical complement pathway abnormality
  • Complement deficiency disease
  • Familial C3B inhibitor deficiency syndrome
  • Oedema of mouth
  • aHUS (atypical haemolytic uraemic syndrome) with complement gene abnormality
  • aHUS (atypical hemolytic uremic syndrome) with anti-factor H antibodies
  • Disorder of complement
  • Complement 4A deficiency
  • C8 binding protein deficiency
  • Complement 2 deficiency
  • Atypical haemolytic uraemic syndrome
  • PLE - Protein-losing enteropathy
  • Combined complement 6 and 7 deficiencies
  • Complement hyperactivation, angiopathic thrombosis, protein losing enteropathy syndrome
  • Immunodeficiency due to MASP-2 deficiency
  • C1 esterase inhibitor [C1-INH] deficiency
  • Oedema of oral soft tissues
  • Complement 5 deficiency
  • Autoimmune angio-oedema
  • Hereditary angioneurotic edema with C1 inhibitor deficiency
  • C3 deficiency
  • Autoimmune urticaria and/or angioedema
  • Complement abnormality
  • C8 (complement component 8) deficiency
  • Hereditary angio-oedema
  • Cutis laxa, acquired type
  • Hereditary C1 esterase inhibitor deficiency - deficient factor
  • Complement 4 deficiency
  • Atypical hemolytic uremic syndrome with anti-factor H antibodies
  • Factor D deficiency
  • Primary CD59 deficiency
  • Factor B deficiency
  • Complement receptor deficiency
  • Hereditary angioedema - type 2
  • aHUS - atypical hemolytic uremic syndrome
  • complement deficiencies
  • Coombs negative haemolytic anaemia
  • Acquired angioneurotic edema with C1 inhibitor deficiency
  • Hereditary angioedema
  • Homologous restriction factor deficiency
  • Hereditary angioneurotic edema
  • Hereditary C1 esterase inactivity
  • Properdin deficiency
  • Complement 8 alpha-gamma deficiency
  • Factor H deficiency
  • aHUS (atypical hemolytic uremic syndrome) with complement gene abnormality
  • Glomerular disease due to complement system disorder
  • Acquired cutis laxa
  • Complement 4 binding protein deficiency
  • CD59 - Cluster of differentiation 59 deficiency
  • CR3 (complement receptor 3) deficiency
  • Complement 8 beta chain deficiency
  • Hereditary angio-oedema - type 1
  • Hereditary angioneurotic edema - type 2
  • Alternative pathway deficiency
  • aHUS (atypical haemolytic uraemic syndrome) with anti-factor H antibodies
  • Complement component 3 deficiency
  • Hereditary angio-oedema - type 2
  • aHUS - atypical haemolytic uraemic syndrome
  • Acquired C1 esterase inhibitor deficiency
  • Acquired angioneurotic oedema with C1Inh (C1 esterase inhibitor) deficiency
  • Acquired angioedema
  • Hereditary angioneurotic oedema - type 1
  • C4AQ0 - Complement 4A deficiency
  • Recurrent Neisseria infection due to factor D deficiency
  • Terminal component deficiency
  • CHAPLE syndrome
  • CHAPLE (complement hyperactivation, angiopathic thrombosis, protein losing enteropathy) syndrome
  • Anaphylotoxin inactivator deficiency
  • Acquired angioneurotic oedema with C1 inhibitor deficiency
  • Complement 8 beta chain dysfunction
  • Complement 5a inhibitor deficiency
  • HAE (hereditary angioedema) with C1Inh (C1 esterase inhibitor) deficiency
  • Immunodeficiency due to mannan binding lectin serine peptidase 2 deficiency
  • Complement receptor 3 deficiency
  • Hereditary Quincke's edema
  • Hereditary angioneurotic oedema
  • Angio-oedema due to disorder of kinin metabolism
  • Atypical haemolytic uraemic syndrome with anti-factor H antibodies
  • Complement 1r deficiency
  • Complement 9 deficiency
  • Hereditary angioneurotic oedema - type 2
  • Immunodeficiency due to MASP-2 (mannan binding lectin serine peptidase 2) deficiency
  • Complement deficiency disease (disorder)
  • Acquired angioedema with C1Inh (C1 esterase inhibitor) deficiency
  • Complement component deficiency
  • Gingival oedema
  • Complement 1s deficiency
  • Hereditary angioedema - type 1
  • Coombs negative hemolytic anemia
  • Complement regulatory factor defect
  • CD55 - Cluster of differentiation antigen 55 deficiency
  • Panniculitis with complement deficiency
  • Hereditary angioneurotic oedema with C1 inhibitor deficiency
  • Hereditary angioneurotic edema - type 1
  • deficiency complement
  • Anemia due to mechanical damage
  • Cutis laxa with complement deficiency
  • Anaemia due to mechanical damage
  • Acquired angio-oedema
  • Kininase I deficiency
  • Complement 1q deficiency
  • Hereditary Quincke's oedema
  • Acquired angioedema due to C1 inhibitor autoantibody
  • Atypical haemolytic uraemic syndrome with complement gene abnormality
  • Decay accelerating factor deficiency
  • Panniculitis due to immunological disorder
  • Edema of mouth
  • Autoimmune angioedema
  • Hereditary C1 esterase inhibitor deficiency - dysfunctional factor
  • Atypical HUS (hemolytic uremic syndrome) with anti-CFH (complement factor H) Ab
  • Autoimmune urticaria and/or angio-oedema
  • Atypical HUS (haemolytic uraemic syndrome) with anti-CFH (complement factor H) Ab
  • Gingival edema
  • HANE - Hereditary angioneurotic edema
  • Edema of oral soft tissues
  • HAE - Hereditary angio-oedema
  • Atypical hemolytic uremic syndrome with complement gene abnormality
  • Acquired angio-oedema due to C1 inhibitor autoantibody
  • C1 esterase inhibitor deficiency - type 2
  • Disorder of glomerulus due to complement system disorder
  • Recurrent bacterial infection
  • Acquired angioneurotic edema with C1Inh (C1 esterase inhibitor) deficiency
  • Complement component 8 deficiency
  • Complement 4B deficiency
  • Complement receptor 1 deficiency
  • C1 esterase inhibitor deficiency - type 1
  • Complement 1q dysfunction
  • Hereditary angioedema with C1Inh (C1 esterase inhibitor) deficiency
  • Angioedema of gingiva due to deficiency of C1 esterase inhibitor
  • Properdin deficiency disease
  • HANE - Hereditary angioneurotic oedema
  • C4BQ0 - Complement 4B deficiency
  • Complement 5 dysfunction
Frequently Asked Questions
What is the ICD-10 code for defects in the complement system?

The ICD-10-CM code for defects in the complement system is D84.1. The full clinical description is "Defects in the complement system". D84.1 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code D84.1 mean?

ICD-10-CM code D84.1 represents “Defects in the complement system”. It is classified under Chapter 3: Diseases of the Blood and Blood-Forming Organs and is a billable/specific code that can be used on a claim.

Is D84.1 a billable code?

Yes, D84.1 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is D84.1 in?

D84.1 is in Chapter 3: Diseases of the Blood and Blood-Forming Organs (codes D50-D89).

What codes cannot be used with D84.1?

D84.1 has Excludes1 notes indicating codes that cannot be used together with it, including: autoimmune disease (systemic) NOS (M35.9); functional disorders of polymorphonuclear neutrophils (D71); human immunodeficiency virus [HIV] disease (B20).

What SNOMED CT codes does D84.1 map to?

D84.1 maps to 69 SNOMED CT concepts: 241955009, 703802001, 19726003, 234604001, 34247008, and 64 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for D84.1?

D84.1 is linked to 2 UMLS Concept Unique Identifiers: C2873848, C0272242. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does D84.1 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like defects in the complement system affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of D84.1?

D84.1 maps to the ICD-11 code: 4A00.1Z (Defects in the complement system, unspecified).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.