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ICD Hub / Diseases of the immune system (D80–D89) / 4A00.10 - Immunodeficiency with an early component of complement deficiency
ICD-11

ICD-11 Codes
for 4A00.10 - Immunodeficiency with an early component of complement deficiency
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Understanding Immunodeficiency with Early Complement Component Deficiency

This condition, identified by the ICD-11 code 4A00.10, is a type of primary immunodeficiency. It arises from defects in the early components of the complement system, which is a crucial part of the innate immune system. The complement system helps the body fight off infections by marking pathogens for destruction and recruiting immune cells. When early components like C1, C2, or C4 are deficient, the body's ability to effectively clear certain types of infections, particularly bacterial ones, is significantly impaired. This ICD-11 code specifically addresses deficiencies affecting the initial pathways of complement activation.

Recognising Symptoms of Early Complement Component Deficiency

Individuals with Immunodeficiency with an early component of complement deficiency often experience recurrent infections. These are typically bacterial in nature and can affect various parts of the body. Common presentations include:
  • Recurrent sinopulmonary infections (e.g., pneumonia, bronchitis)
  • Sepsis (a life-threatening bloodstream infection)
  • Meningitis
  • Skin infections
Increased susceptibility to autoimmune diseases may also be observed in some cases. The severity and frequency of infections can vary depending on the specific complement component affected.

Factors Contributing to Early Complement Component Deficiency

The primary cause of Immunodeficiency with an early component of complement deficiency is genetic. These are inherited disorders, often passed down in an autosomal recessive pattern, meaning an individual must inherit a faulty gene from both parents to develop the condition. While specific genetic mutations lead to deficiencies in C1q, C1r, C1s, C2, or C4, the underlying risk factor is the inheritance of these genetic predispositions. There are no known environmental risk factors that directly cause these specific complement deficiencies.

Diagnosing ICD-11 Code 4A00.10

Diagnosis of Immunodeficiency with an early component of complement deficiency typically involves a combination of clinical evaluation and laboratory testing. A healthcare provider will assess the patient's history of recurrent infections. Specific laboratory tests are crucial for confirming the diagnosis. These include:
  • Total hemolytic complement assays (e.g., CH50) to assess the overall function of the classical complement pathway.
  • Specific assays to measure the levels and function of individual complement components such as C1q, C1r, C1s, C2, and C4.
Genetic testing may also be employed to identify the specific gene mutations responsible for the deficiency. This ICD-11 code for complement deficiency aids in precise medical record classification.

Managing Immunodeficiency with Early Complement Component Deficiency

Management of Immunodeficiency with an early component of complement deficiency focuses on preventing and treating infections. Key considerations include:
  • Prompt and aggressive treatment of bacterial infections with appropriate antibiotics.
  • Prophylactic antibiotic therapy may be prescribed to reduce the risk of recurrent infections.
  • Vaccinations are important, though their effectiveness can sometimes be reduced in individuals with compromised immune systems.
  • In specific cases, such as C1 inhibitor deficiency (though this is a different category, it highlights complement-related therapies), replacement therapy with C1 inhibitor concentrate might be used.
Patients should seek immediate medical advice for any signs of infection.

Alternative Names for ICD-11 Code 4A00.10

This condition is also known by several other terms, reflecting its impact on the complement system. These include:
  • Deficiency of complement initial pathway
  • Complement component C1q deficiency
  • Complement component C1r/C1s deficiency
  • Complement component C2 deficiency
  • Complement component C4 deficiency
Understanding these synonyms is helpful for comprehensive medical coding and documentation.

Frequently asked questions

What is the ICD-11 classification meaning of 'Immunodeficiency with an early component of complement deficiency'?
This ICD-11 classification denotes a primary immunodeficiency characterized by a deficiency in one or more of the early components of the complement system.
How is 'Immunodeficiency with an early component of complement deficiency' coded in ICD-11?
This condition is coded using the specific ICD-11 code 4A00.10, which falls within the broader category of Primary immunodeficiencies.
What is the purpose of the ICD-11 classification for 'Immunodeficiency with an early component of complement deficiency'?
The ICD-11 classification of this condition serves as the international standard for systematic recording, reporting, analysis, interpretation, and comparison of mortality and morbidity data.
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