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2025 ICD-10-CM code D84.1

Defects in the complement system, such as C1 esterase inhibitor [C1-INH] deficiency.

Use additional codes to specify any associated manifestations, such as recurrent infections (e.g., pneumonia, meningitis) or angioedema (e.g., swelling of the tongue, larynx, or intestines).If a specific complement protein deficiency is identified (e.g., C3 deficiency), it should be coded accordingly using the appropriate subcategory under D84.

Medical necessity for services related to complement deficiencies is established by the presence of signs, symptoms, or laboratory findings consistent with a complement disorder.The documentation should clearly link the patient's condition to the need for specific diagnostic tests, treatments, or consultations. For example, in the case of HAE, the frequency and severity of angioedema attacks justify the medical necessity of C1 inhibitor therapy.

Clinicians, including immunologists, allergists, and geneticists, are responsible for diagnosing and managing patients with complement deficiencies.Diagnosis typically involves a thorough medical history, physical examination, and laboratory tests, such as complement assays and genetic testing.Treatment varies depending on the specific defect and may include prophylactic antibiotics, immunomodulatory therapies, or targeted treatments like C1 inhibitor concentrate for HAE.Patient education regarding infection prevention, early symptom recognition, and disease management is essential.

In simple words: Defects in the complement system mean that part of your immune system isn't working correctly.This can make you more likely to get infections or develop certain diseases.One example is a condition called hereditary angioedema, where people experience episodes of swelling, particularly in the face, throat, and abdomen.

This code encompasses various inherited or acquired defects affecting the complement system, a crucial part of the immune system responsible for defending against infections and maintaining homeostasis.These defects can lead to increased susceptibility to infections, autoimmune diseases, or other related conditions.A prime example is C1 esterase inhibitor deficiency, which can cause hereditary angioedema (HAE).Symptoms of complement deficiencies vary based on the specific defect but may include recurrent infections, autoimmune manifestations, or angioedema.

Example 1: A patient presents with recurrent bacterial infections and is found to have a deficiency in C3, a key component of the complement system.The patient is diagnosed with C3 deficiency and started on prophylactic antibiotics., A patient experiences episodes of severe swelling in the face and throat, triggered by stress or minor trauma.Laboratory testing reveals low levels of C1 esterase inhibitor, confirming the diagnosis of hereditary angioedema.The patient is prescribed C1 inhibitor concentrate for acute attacks and prophylactic therapy to prevent future episodes., A child experiences recurrent episodes of meningitis and is found to have a genetic defect in a complement protein. The diagnosis is a complement deficiency, and preventive measures, along with targeted therapies, are discussed with the family.

Thorough documentation of medical history, family history of similar conditions, physical examination findings, including details of infections or angioedema episodes, and laboratory results, such as complement levels (C3, C4, CH50, AH50) and genetic testing results, are crucial.Any administered treatments and the patient's response to therapy should also be documented.

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