ANCA Vasculitis

What is Vasculitis?

Vasculitis is inflammation (“-itis”) of blood vessels (vasculum = vessel). Inflammation is caused by white blood cells attacking something in a tissue. When “-itis” is at the end of a diagnosis, it means that swelling is involved in the disease. There are many different causes of vasculitis. Some vasculitis is caused by infection of vessels (as in Rocky Mountain spotted fever). Another cause of vasculitis is autoimmunity.

What is Autoimmune Vasculitis?

Autoimmune vasculitis is caused when the immune system causes vasculitis. Normally, the immune system defends against foreign invaders (germs). It  causes white blood cells to attach the invaders and swelling occurs. In an autoimmune disease, the immune system causes white blood cells to attack one’s own self (autos = self).  In different autoimmune diseases, white blood cells attack different cells and tissues of the body. In autoimmune vasculitis white blood cells attack one’s own blood vessel walls causing swelling of the vessels.

What is ANCA Vasculitis?

ANCA vasculitis is a type of autoimmune swelling caused by autoantibodies. Normal antibodies are parts in the blood that are produced by the immune system to fight germs. Autoantibodies are abnormal antibodies that attack one’s own cells and tissues (autos = self). ANCAs are autoantibodies that attack the inside (cytoplasm) of a certain type of white blood cells called neutrophils. ANCA stands for Anti-Neutrophil Cytoplasmic Autoantibody. When ANCAs attack these neutrophils, they cause the white blood cells to attack the walls of small vessels in different tissues and organs of the body. This causes vasculitis. For example, vasculitis in the skin causes red spots. In the lungs or nose it causes bleeding. In the nerves it causes tingling or weakness. In the eyes it causes redness and itching. In the kidneys vasculitis causes leaking of blood and protein into the urine and kidney failure.

Blood Vessel and Blood Cells

Below is an illustration of a piece cut from a small blood vessel. The blood vessel contains blood that is made of red cells and white cells floating in a liquid called plasma. The inside surface of the vessel is lined by cells that sit on a thin casing.

Blood vessel 

ANCA vasculitis is caused when ANCAs attack neutrophils. This causes the white blood cells to attack vessel walls, which creates swelling.

ANCA Vasculitis - Vessel wall

Shown below on the left is how a normal small vessel would look under the microscope. On the right is how the vessel looked after it was injured by ANCA VASCULITIS. Compare this to the earlier illustration  to understand what has happened.

ANCA Vasculitis - inflammation

Autoantibodies

Most patients with ANCA vasculitis have autoantibodies specific for perinuclear (P-ANCA) or cytoplasmic (C-ANCA) antibodies. C-ANCA is usually directed against proteinase 3 (PR3) and P-ANCA is usually directed again myeloperoxidase (MPO). The images below show what C and P-ANCA look like under a microscope when they are stained by a special chemical.

ANCA Vasculitis-Patterns

What are the different types of ANCA Vasculitis?

In different people, ANCA vasculitis attacks different organs. It may  have different disease signs. These are used to make the diagnosis of a specific form of ANCA vasculitis.

  • Renal limited vasculitis or ANCA glomerulonephritis: The blood vessel swelling is causing capillary swelling. No other organs are affected.
  • Microscopic polyangiitis: The swelling is causing injury to blood vessels in multiple tissues at the same time. It can be seen in the kidneys, skin, nerves, and lungs.
  • Granulomatosis with polyangiitis -GPA- (Formerly Wegener's granulomatosis): The swelling happens with another kind of swelling called granulomatous inflammation. This often affects the lung, sinuses, nose, eyes or ears.
  • Eosinophilic granulomatosis with polyangiitis -EGPA- (Formerly Churg-Strauss Syndrome): There is swelling, granulomatous inflammation, as well as asthma.

ANCA Vasculitis - Purpura

What is ANCA Glomerulonephritis?

ANCA can cause white blood cells (neutrophils) to attack the capillaries in the filtering units (glomeruli) of the kidney. This swelling of glomeruli is called glomerulonephritis. The destruction of the glomerular capillaries causes blood and proteins to spill into the urine. This causes the kidney to stop functioning. Glomerulonephritis often occurs in patients with ANCA disease.

ANCA Vasculitis - Glomerulonephritis Diagram

Who gets vasculitis?

Vasculitis is very rare, occurring in 1 out of 50,000 people. The average vasculitis patient is 55 years old. Some very young children and elderly people can also develop vasculitis. Vasculitis affects whites more than minorities. It affects men and women equally.

What does it affect, and what are the symptoms?

Vasculitis can affect any part of the body. The chart below names parts of the body affected by vasculitis and the doctor who specializes in that body part:

Affected body partDoctor
Kidney Nephrologist
Joints Rheumatologist
Lungs Pulmonologist
Skin Dermatologist
Sinus/Nose ENT
Trachea ENT
Nerves Neurologist
Gastrointestinal Tract Gastroenterologist
Eyes Opthalmologist

The symptoms of vasculitis include flu-like symptoms such as fever, body aches, joint and muscle pain. There can also be reduced appetite, and weight loss. Symptoms can appear in the particular body part that is affected by the disease. The chart below explains some common symptoms that are specific to the affected body part. 

Affected Body PartSymptoms
Kidney Brown, tea-colored urine from blood leaking into the urine
Joints Joint pain or swelling
Lungs Heavy cough, often mistaken for pneumonia, coughing up blood
Skin Red or purple spots , itching, hives, rash
Sinus/Nose Runny nose that get worse and worse, nose pain, trouble breathing through nose
Trachea Shortness of breath
Gastrointestinal Tract Stomach pain, or blood in stool
Eyes Red, painful eyes, blurry vision, headaches
Ears Hearing problems or hearing loss

How long does vasculitis last?

Vasculitis can be a short-term illness that is cured by treatment. It can also be a long-term disease that never completely goes away. Many people with long-term vasculitis live long, healthy lives. They can enjoy long periods when the disease has few or no symptoms. Regular check-ups are very important for patients who have been diagnosed with vasculitis because there is always a risk that the disease will come back.

What types of tests detect vasculitis?

Doctors use a combination of lab tests and physical exams to diagnose vasculitis. These tests usually focus on the area of the body that is affected. Many times patients need specialists to look at a specific area. Below are some tests that are often used to diagnose vasculitis:

  • A blood test to check for ANCA
  • Blood and urine tests to check to see if vasculitis is affecting the kidneys
  • Chest x-rays or CT scan to check for lung problems
  • Endoscopic exam by an ENT to check the ears, nose, and throat
  • A small tissue sample, called a biopsy, may be taken from an affected area for a lab to look at more closely

How is it treated? 

The treatment of vasculitis can be complicated. It should always involve specialists for each affected body system.

Doctors can use different approaches to try to reduce the signs  of the disease. At the same time, dangerous or uncomfortable side effects from the treatment should be minimized. The goal of the treatment is to reduce the swelling to protect organs from damage without hurting the immune system. Other infections can spread when the immune system does not work well.

Exact treatment depends on:

  • The type of vasculitis
  • How serious the disease is
  • How many organ systems are affected

Treatment options include:

  • Corticosteroids: (prednisone, methyl prednisone, or Medrol) A type of drug that can be given by IV or in pill form. They help reduce swelling and slow down the autoimmune response in vasculitis.
  • Immunosuppressive Drugs: (cyclophosphamide, cyclosporine, azathioprine, mycophenolate mofetil, or Rituximab) Drugs that reduce the immune system's tendency to attack itself.
  • Antibiotics: (trimethoprim/sulfamethoxazole) Drugs that kill foreign infections that can live  in the nose or in patients with a weak immune system Plasmapheresis: A treatment that removes the ANCA from the blood. This treatment is usually only for patients with bleeding in the lungs or kidney failure.

What should I do if I have vasculitis?

Learning that you have a rare chronic disease can be scary, stressful, and overwhelming. Healthcare providers will be sharing lots of information about your condition and the treatment options. One of the most helpful things you can do is to have a trusted friend or family member with you at your appointments. Ask them to take notes and help you ask questions. This will reduce the worry that you might forget a question, or not remember some important information your doctor tells you. Here are some questions that many patients find helpful to ask their doctor:

  • What is my diagnosis?
  • What stage is my disease?
  • What are my treatment choices?
  • Which option do you recommend?
  • Why?
  • What are the chances that the treatment will be successful?
  • What are the risks and side effects of treatment?
  • How long will the treatment last?
  • Will I have to change my normal activities?
  • What will treatment cost?

Kidney transplant in ANCA vasculitis

In some patients, even with treatment, involvement of the kidney with ANCA vasculitis will lead to kidney failure. Fortunately, kidney transplant is a treatment option for these patients.

Visit this page in the Kidney Health Library for general information about kidney transplant.

Will the ANCA disease come back in my kidney transplant?

About 10-15% of patients will have symptoms of ANCA disease after a kidney transplant. This is called recurrent disease. Not all return symptoms affect the kidney, but some will. It is important that ANCA disease be inactive prior to getting a kidney transplant. This reduces the chance that the ANCA disease will come back after the transplant. 

Generally, ANCA disease recurrence will occur between 2.5 and 4 years after transplant. But it can occur any time.

Is there any treatment for ANCA disease that comes back in a transplant?

Treatments are similar to those for patients with ANCA disease in their original kidneys. They include corticosteroids and immunosuppressive drugs. These are different from the ones taken to prevent kidney transplant rejection.

If the ANCA disease comes back, will it cause me to lose my kidney transplant?

Recurrent ANCA disease can rarely cause loss of a transplanted kidney. If you look at all patients with ANCA disease 10 years after their transplant, about 8% of them will have lost their kidney due to recurrent ANCA disease. 

A patient with ANCA disease who gets a kidney transplant can expect a similar life span and kidney transplant survival as a transplant patient who has kidney failure from another disease.

The Vasculitis Foundation

The Vasculitis Foundation was created to help the isolation that vasculitis patients and their families experience. They provide support, awareness, and research for all types of vasculitis. 

E-mail: vf@vasculitisfoundation.org

The North Carolina/Raleigh Vasculitis Support Group

Clinical Trials

Learn about clinical trials and observational studies at the UNC Kidney Center

ClinicalTrials.gov provides regularly updated information about federally and privately supported clinical research. It includes information about a trial's purpose, who may participate, locations, and phone numbers for more details.

CenterWatch Clinical Trials Listing Service: this site gives general information about clinical trials. It includes listings of thousands of industry-sponsored clinical trials that are actively looking for patients in the U.S. and internationally.