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ANCA Vasculitis - Types, Causes, Symptoms, and Treatment

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ANCA vasculitis is an autoimmune condition of the blood vessels. Read this article to know more.

Medically reviewed by

Dr. Mohamad Ali Rida

Published At January 19, 2023
Reviewed AtJanuary 19, 2023

What Is ANCA Vasculitis?

ANCA vasculitis is an autoimmune condition that affects the blood vessels. ANCA can be expanded to anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). This condition occurs when certain antibody receptors bind to immune cells called neutrophils. This binding can over-activate the immune system leading to a series of reactions. Neutrophils, the immune cells, affect the body's small blood vessels. This can cause inflammation of the blood vessels. Inflammation is the body’s defense mechanism that helps remove the threat to the body. The effect of this reaction can be felt in various parts of the body, such as the stomach, kidneys, skin, and intestines. The symptoms of AAV can vary depending on its location. This immune system overreaction is believed to be genetic and environmentally acquired. Depending on the symptoms and other factors, such as microscopic observation, this condition can be classified into three main sub-categories.

What Are the Types of AAV?

ANCA vasculitis can be subdivided into three main categories. This classification is achieved based on the symptoms coupled with microscopic findings. This classification helps in the treatment of the condition. The classification of AAV is as follows:

  • Microscopic Polyangiitis (MPA): MPA patients ordinarily experience various side effects like kidney conditions, skin conditions, and nerve injury. This condition is frequently associated with recurrent fever and weight loss. MPO-ANCAs is the antibody that is associated with MPA.

  • Granulomatosis With Polyangiitis (GPA): This condition is characterized by the formation of granulomas in the blood vessels. Granulomas are a group of immune cells that attach to the lining of the blood vessels. This type of AAV was also known as Wegener's granulomatosis. Patients with this condition have damage to the blood vessels of various organs such as kidneys, lungs, and respiratory organs.

  • Eosinophilic Granulomatosis With Polyangiitis (EGPA): This category of AAV affects only the gastrointestinal and respiratory systems. Other organs can be affected, but it is very rare. Clusters of immune cells called granulomas also cause this condition. One variation in this type of AAV is that the immune clusters are primarily made of eosinophils, not neutrophils. Eosinophils are also a type of immune cell. This condition presents with asthma-like symptoms hence its diagnosis may be difficult. Accurate diagnosis of this condition is possible only through microscopic examination.

What Is the Occurrence of AAV?

This condition is not very uncommon. AAV is known to affect at least one out of every 50,000. This condition affects both men and women equally. More cases were reported in the middle-aged population. While considering racial predilection, this condition most commonly affects the white population.

What Is Meant by Autoimmune Condition?

In absolutely healthy individuals, the immune framework recognizes unfamiliar trespassers and produces antibodies that kill them before they can cause any harm to the body. An autoimmune condition occurs when the insusceptible framework produces unusual autoantibodies that target sound cells and tissue. In AAV, autoantibodies called ANCAs are found at raised levels and attached to immune cells called neutrophils. This causes the accentuation of neutrophils, which then, at that point, are attached to the lining of the veins and discharge harmful granules. This activates the immune response leading to damage to the vasculature, causing swelling. Antibodies focusing on the proteinase 3 (PR3) or the myeloperoxidase (MPO) proteins on neutrophils are the most widely recognized ANCAs in individuals with AAV, each subsequent in unmistakable sickness signs. Rarely can ANCAs target other proteins as well.

What Are the Causes of AAV?

There is no single reason for the occurrence of this condition. This condition can be caused by a single source or can occur due to a mixture of various factors. The causes can be genetic, environmental, or even an infection. The factors causing AAV include the following:

  • Genetic: Genetic factors have the strongest association with AAV. The alterations in genetic factors such as the MHC (major histocompatibility complex) are believed to cause this condition. Any alterations to MHC can alter the protein structure of the antibodies produced. These alterations to the MHC can happen through familial inheritance or even mutations.

  • Silica Inhalation: Silica is a metal found in various places and materials. Inhalation of silica can affect a person’s respiratory system. It is believed that silica, when inhaled in greater amounts, can stimulate the occurrence of AAV. Silica can be found in asbestos sheets, pesticides, and industrial chemicals.

  • Cocaine: Prolonged use of cocaine can have various detrimental effects on health. AAV is one of them. The exact link between cocaine misuse and AAV is not completely understood. However, cocaine users were found to be affected by AAV.

  • Infections: Certain bacterial or viral infections have been known to be a prelude to AAV. After the infection by an organism, it is known that the body is on high vigil. This can cause the immune system to overreact. This can, in turn, lead to autoimmune conditions such as AAV.

What Are the Symptoms of AAV?

AAV is known to affect various parts of the body. Hence, the symptoms of AAV depend on the organ affected. Therefore, the various symptoms of AAV according to the system affected may include the following:

Renal Symptoms: When AAV affects the renal system, the following effects may be seen in the kidneys.

  1. Blood in the urine.
  2. Change the urine color to brown.
  3. Foamy urine.
  4. Elevated blood pressure due to the effect on the renal vasculature.

Respiratory Symptoms: When AAV affects the respiratory system, it may cause trachea inflammation. The symptoms may include difficulty breathing, changes in voice, continuous cough, sinus infections, ear infections, and discharges from the nose.

Nervous System Symptoms: Nervous system symptoms may include

  1. Weakness of the muscles.
  2. Tingling sensation.
  3. Numbness in the affected region.
  4. Pricking-like sensation.
  5. Frequent headaches and dizziness.
  6. Seizures are also possible in some cases.

Gastrointestinal Symptoms: When AAV affects the stomach, the symptoms may include:

  1. Stomach aches and cramps.
  2. Nausea and vomiting.
  3. Watery stools or diarrhea.
  4. Blood in feces.
  5. Weight loss without any apparent reason.

Muscular Symptoms: Muscular symptoms may include muscular weakness, cramps, and severe pain.

How Is AAV Diagnosed?

  • A doctor can diagnose AAV by reviewing the physical and laboratory findings. The lab tests help identify the type of AAV that aids in its diagnosis. Blood and urine analyses will be performed to obtain a definitive result. Sometimes the doctor may even perform a biopsy of the vascular tissues to check for granuloma formation.

  • The doctor may suggest imaging studies to substantiate the blood and urine analyses. The imaging studies may include X-rays and CT (computed tomography) scans. Depending on the need, the patient may also require other specific tests, such as endoscopy or bronchoscopy.

How Is AAV Treated?

  • While there is no single solution for AAV, various medications are accessible to deal with the condition. These decrease and, at times, resolve the vascular inflammation and the associated swelling.

  • Patients ordinarily go through two treatment periods. The first one usually aims at resolving the condition at hand. The second treatment is a maintenance treatment that aims at preventing recurrence.

  • The medicines utilized greatly rely upon sickness seriousness and subtype, yet most are intended to bring down the overactive immune system. This type of treatment usually involves the usage of steroids such as Methotrexate, Corticosteroids, and Rituximab.


AAV is one such medical condition that does not have any specific causes or symptoms. It is believed to be caused due to a mix of genetic and environmental factors. It affects various body systems, such as the lungs, gastrointestinal, and kidneys. Most of its symptoms can overlap with the symptoms of various other conditions. Hence, a medical opinion should be obtained soon if any of the above-mentioned symptoms are observed.

Frequently Asked Questions


What Is the Cause of Anca Vasculitis?

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, or ANCA vasculitis, is a collection of autoimmune disorders that result in blood vessel inflammation. Although the precise etiology of ANCA vasculitis is unknown, a mix of immune system failure, environmental stressors, and genetic susceptibility is thought to be the cause.


Is Recovery Possible From Anca Vasculitis?

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, or ANCA vasculitis, is a collection of autoimmune illnesses. With the right medical care and management, recovery from ANCA vasculitis is achievable, although the degree of recovery will vary based on the severity of the condition and personal circumstances.


What Is the Treatment for Anca Vasculitis?

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, or ANCA vasculitis, is a collection of autoimmune illnesses. With the right medical care and management, recovery from ANCA vasculitis is achievable, although the degree of recovery will vary based on the severity of the condition and personal circumstances.


What Is the Vasculitis Death Rate?

Vasculitis death rates can be substantial in extreme cases, but they vary greatly depending on the kind, severity, and individual circumstances. Results can be enhanced by early diagnosis and rapid medical intervention. Seeking advice and a more accurate assessment from a healthcare professional is imperative.


Which Organs Are Affected by Anca Vasculitis?

Small blood vessels, particularly those in the kidneys, lungs, and upper respiratory tract, are the main target of ANCA vasculitis; however, it can also affect other organs.


What Is the Prevalence of Anca Vasculitis?

An estimated 20 to 30 cases of ANCA vasculitis are reported per million people, a relatively low prevalence. However, prevalence might differ depending on the population and geography.


Does Anca Vasculitis Run in Families?

Unlike several other disorders, ANCA vasculitis is not usually seen as a hereditary or genetic disease. Nonetheless, some data points to a genetic predisposition, which means that a person may be more susceptible to the disease if they have particular genetic markers or variations. It is not inherited in a Mendelian manner, although it can affect several family members simultaneously (when it follows a clear inheritance pattern, like some hereditary disorders). ANCA vasculitis is also greatly influenced by immune system interactions and environmental variables. As a result, unlike many genetic disorders, it is not thought to run in families; nonetheless, it may emerge as a result of a mix of genetic and environmental factors.


What Is the Most Recent Anca Vasculitis Treatment?

Immunosuppressive therapies, such as corticosteroids and other immune-suppressive pharmaceuticals, are commonly used to treat ANCA vasculitis. Treatments like cyclophosphamide and rituximab are frequently utilized. New studies and treatments are still being developed, though.


What Is the Anca-Associated Vasculitis Maintenance Treatment?

Medications that assist in minimizing the negative effects of long-term immunosuppression and help avoid disease relapses are frequently used in maintenance treatment for ANCA-associated vasculitis. Low-dose corticosteroids and immunosuppressive medications such as methotrexate or azathioprine are common choices. It is also possible to employ more recent medications, including rituximab. Several maintenance therapies may be chosen depending on the patient's kind of ANCA vasculitis and how well they respond to induction therapy.


Is Vasculitis Anca Permanent?

Although ANCA-associated vasculitis is a chronic illness, medication is frequently effective in managing it. It may go into remission with the right treatment, but it can also recur occasionally. Controlling the condition and its symptoms usually requires long-term monitoring and treatment.


How Much Time Does Anca Vasculitis Treatment Take?

The length of treatment for ANCA vasculitis varies greatly based on the patient's response to the medication, the nature and severity of the illness, and other variables. The length of treatment might vary from a few months to several years. Maintenance therapy may be required to avoid recurrence for a considerable period of time in certain situations. A healthcare professional will decide each patient's treatment plan and its duration.


Can Someone With Anca Vasculitis Lead a Normal Life?

With the right care and therapy, many people with ANCA vasculitis can lead typical lives. However, the degree of the illness and how well it responds to treatment can affect day-to-day functioning. While some people may have continuous difficulties, others may go through times of remission during which they feel good. For people with ANCA vasculitis, maintaining the illness and enhancing the quality of life depend heavily on regular medical check-ups and strict adherence to treatment regimens.


What Consequences Does Anca Vasculitis Have in the Long Run?

Damage to impacted organs like the kidneys, lungs, and blood vessels might result from ANCA vasculitis over the long run, potentially resulting in chronic health problems. The severity of these effects might vary, and managing these disorders frequently requires continued medical treatment.


Which Foods Are Best to Avoid When an Individual Has Anca Vasculitis?

Although specific dietary advice may differ, it's generally a good idea for people with ANCA vasculitis to think about cutting back on or avoiding things like processed meals, a lot of salt, and sugar that might cause inflammation. Following a balanced diet that prioritizes whole grains, fruits, vegetables, lean meats, and healthy fats may be advantageous. Speaking with a medical professional or a licensed dietician is advised for individualized nutritional advice.


How Is Daily Living Impacted by Vasculitis?

Depending on the degree and exact nature of the illness, vasculitis can have various effects on day-to-day functioning. It can cause symptoms including soreness, exhaustion, and discomfort, making it difficult for the person to work out, work, or do their everyday business. Medical procedures, follow-up visits, and possible drug side effects can also impact daily living. Managing vasculitis frequently necessitates meticulous collaboration with healthcare providers to preserve the best possible quality of life.

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Dr. Mohamad Ali Rida
Dr. Mohamad Ali Rida



anca vasculitis
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