Discover free eBooks, guides and med spa templates on our new resources page

Book a demo
☰
Why us
ICD Hub / Diseases of the immune system (D80–D89) / 4A00.11 - Immunodeficiency with a late component of complement deficiency
ICD-11

ICD-11 Codes
for 4A00.11 - Immunodeficiency with a late component of complement deficiency
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
Use Code

Understanding Immunodeficiency with a Late Component of Complement Deficiency

Immunodeficiency with a late component of complement deficiency, identified by the ICD-11 code 4A00.11, is a primary immunodeficiency disorder. This condition arises from anomalies in the later stages of the complement system, specifically involving complement components C5, C6, C7, C8, or C9. The complement system is a vital part of the innate immune response, and its deficiency in these late components significantly impairs the body's ability to fight certain infections. Individuals with this diagnosis are particularly susceptible to infections caused by Neisseria bacteria, which can lead to serious conditions such as meningitis. While there is no definitive cure, the prognosis is generally considered favourable with appropriate medical management.

Factors Contributing to Late Complement Component Deficiency

The primary cause of immunodeficiency with a late component of complement deficiency (ICD-11 code 4A00.11) is a hereditary anomaly affecting one or more of the terminal complement pathway proteins: C5, C6, C7, C8, or C9. These genetic variations disrupt the complement cascade's ability to form a membrane attack complex (MAC), which is crucial for lysing certain pathogens. As a primary immunodeficiency, it is inherited, meaning individuals are born with the genetic predisposition.

Recognising the Signs of Late Complement Component Immunodeficiency

Individuals diagnosed with immunodeficiency with a late component of complement deficiency (ICD-11 code 4A00.11) often present with a history of recurrent infections. The most significant risk is associated with Neisseria species, leading to conditions such as invasive meningococcal disease, commonly known as meningitis. Disseminated gonococcal infections can also occur. These infections may be severe and require prompt medical attention. The presentation can vary, but a pattern of severe bacterial infections, particularly those caused by Neisseria, is a key indicator.

Diagnostic Approaches for Late Complement Component Deficiency

Diagnosing immunodeficiency with a late component of complement deficiency (ICD-11 code 4A00.11) involves a comprehensive evaluation of the immune system. Initial diagnostic steps typically include functional screening tests of the complement pathways, such as the CH50 assay for the classical pathway and the AH50 assay for the alternative pathway. If these screens indicate a deficiency, further specific testing is performed to quantify the levels of individual complement components, such as C5 through C9. Genetic testing may also be employed to identify the specific mutation responsible for the deficiency.

Accurate Coding for Late Complement Component Immunodeficiency

For accurate medical record-keeping and billing, the ICD-11 code 4A00.11 is used for Immunodeficiency with a late component of complement deficiency. This code falls under the broader category of Primary immunodeficiencies due to disorders of innate immunity (4A00). Specific documentation should include the patient's history of recurrent Neisseria infections, meningitis, or other relevant clinical findings. Terms such as terminal complement pathway deficiency or late complement component deficiency may be used in clinical notes. This ICD-11 code for immunodeficiency is essential for precise classification.

Managing Immunodeficiency with a Late Component of Complement Deficiency

While there is no cure for immunodeficiency with a late component of complement deficiency (ICD-11 code 4A00.11), management focuses on preventing and effectively treating infections. This often involves the judicious use of antibiotics, which may be prescribed prophylactically for individuals at high risk or to treat acute infections promptly. Vaccinations are a critical component of management, with particular emphasis on immunisation against Neisseria meningitidis. Treatment plans are tailored to the individual patient's specific deficiency and clinical presentation, requiring ongoing care from healthcare professionals.

Frequently asked questions

What is the ICD-11 classification meaning of 'Immunodeficiency with a late component of complement deficiency'?
ICD-11 code 4A00.11 classifies an immunodeficiency disorder characterized by a deficiency in the late-acting components of the complement system.
How is the specificity of the complement deficiency addressed when using ICD-11 code 4A00.11?
The code 4A00.11 denotes an immunodeficiency with a late component of complement deficiency. Further detailed specification of the exact complement components involved would be managed through additional coding or context as per ICD-11's post-coordination rules, if applicable.
What does the ICD-11 code 4A00.11 represent in terms of its clinical classification?
In ICD-11, code 4A00.11 represents a specific category within immunodeficiencies, highlighting a deficiency related to the late components of the complement system.
×