Understanding ANCA Vasculitis: ICD-10 Codes And Diagnosis

What is ANCA Vasculitis ICD 10?

ANCA Vasculitis, also known as Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated Vasculitis, is a group of rare autoimmune diseases that cause inflammation of the blood vessels. These diseases are characterized by the presence of ANCAs in the blood, which are antibodies that mistakenly attack the body’s own tissues, leading to inflammation and damage to the blood vessels. ANCA Vasculitis can affect various organs in the body, including the kidneys, lungs, skin, and nerves.

Code Information

The ICD-10 code for ANCA Vasculitis is M31.3. This code is used to classify this type of vasculitis in medical records and for billing purposes. It is important for healthcare providers to accurately code for ANCA Vasculitis in order to ensure proper diagnosis and treatment of the condition.

Diagnostic Related Groups (MS-DRG)

ANCA-associated vasculitis  Nature Reviews Disease Primers
ANCA-associated vasculitis Nature Reviews Disease Primers

ANCA Vasculitis falls under MS-DRG 822, which includes diseases of the skin and subcutaneous tissue with major complications or comorbidities. This DRG is used by Medicare and other payers to classify patients with ANCA Vasculitis and determine reimbursement rates for their care.

Convert to ICD-9 Code

The ICD-9 code for ANCA Vasculitis is 446.4. This code was used prior to the implementation of ICD-10 and is no longer in use for billing purposes. Healthcare providers should ensure they are using the correct ICD-10 code, M31.3, for ANCA Vasculitis.

Code History

ANCA-Associated Vasculitis Management in the United States: Data
ANCA-Associated Vasculitis Management in the United States: Data

ANCA Vasculitis was first classified in the ICD-10 coding system in 2016. Prior to that, it was categorized under other types of vasculitis codes. The creation of a specific code for ANCA Vasculitis has helped improve the accuracy of diagnosis and treatment for patients with this condition.

Approximate Synonyms

Some approximate synonyms for ANCA Vasculitis include Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA), and Microscopic Polyangiitis (MPA). These terms may be used interchangeably with ANCA Vasculitis in medical literature and discussions.

Clinical Information

ANCA-associated vasculitis  Nature Reviews Disease Primers
ANCA-associated vasculitis Nature Reviews Disease Primers

ANCA Vasculitis is a complex disease that can present with a wide range of symptoms and complications. The most common organs affected by ANCA Vasculitis are the kidneys and lungs, leading to symptoms such as hematuria (blood in the urine), proteinuria (protein in the urine), cough, shortness of breath, and chest pain. Other symptoms may include skin rashes, joint pain, and nerve damage.


The exact cause of ANCA Vasculitis is unknown, but it is believed to be triggered by a combination of genetic and environmental factors. The presence of ANCAs in the blood is thought to play a central role in the development of the disease, as these antibodies target and damage the blood vessels, leading to inflammation and tissue damage.


ANCA Vasculitis  UNC Kidney Center
ANCA Vasculitis UNC Kidney Center

The symptoms of ANCA Vasculitis can vary depending on the organs affected and the severity of the disease. Common symptoms may include fatigue, fever, weight loss, joint pain, skin rashes, and nerve pain. Severe cases of ANCA Vasculitis can lead to organ failure and life-threatening complications.


Diagnosing ANCA Vasculitis can be challenging, as the symptoms can mimic other diseases and the presentation of the disease can vary from patient to patient. Healthcare providers may use a combination of blood tests, imaging studies, and tissue biopsies to confirm a diagnosis of ANCA Vasculitis. Testing for the presence of ANCAs in the blood is a key component of the diagnostic process.


Treatment for ANCA Vasculitis typically involves a combination of medications to suppress the immune system and reduce inflammation. Corticosteroids, immunosuppressants, and biologic therapies may be used to manage symptoms and prevent further damage to the blood vessels. In some cases, surgery may be necessary to repair damage to organs such as the kidneys or lungs.


ANCA Vasculitis is a rare autoimmune disease that can have serious consequences if not properly diagnosed and treated. Healthcare providers should be familiar with the

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