Vasculitis and kidney disease

What is vasculitis?

Vasculitis is a group of autoimmune diseases that causes inflammation of the blood vessels. An autoimmune disease causes your body's immune system to mistakenly attack your own tissues. In some forms of vasculitis, a part of your immune system (antibodies) attacks another part of your immune system, (white blood cells). Antibodies are protective proteins produced by your immune system. They attach to foreign substances, like germs or viruses also known as antigens in order to eliminate these foreign substances from your body. In vasculitis, your antibodies mistake your white blood cells for foreign substances. This then causes the white blood cells to release harmful chemicals that cause damage to the blood vessels.  Blood vessels are the tubes that carry blood throughout your body.

Vasculitis causes inflammation of blood vessels, leading to damage. When your blood vessels are inflamed, they can become weak, narrowed or scarred, which makes it harder for blood to get through to organs and tissues. Less blood flow can lead to damage in those organs and tissues.

People with vasculitis usually have periods when their symptoms go away (called remission) and periods when their symptoms get worse or if they have new symptoms (often called a relapse). Patients with vasculitis commonly have one or more relapses over time, but a few patients may only have active symptoms of vasculitis only once.

What are the different types of vasculitis?

There are over 20 different types of vasculitis. Each type affects different parts of the body, depending on which blood vessels are inflamed and damaged.  

Who can get vasculitis?

Anyone can get vasculitis. However, some types of vasculitis may be more common in people of certain ages or ethnic groups. For example, a type of vasculitis called Kawasaki disease mainly affects young children.

What causes vasculitis?

Doctors do not always know the cause of vasculitis. When doctors do not know the cause, it is called primary vasculitis.  

When doctors know the cause, it is called secondary vasculitis. Specific types of vasculitis may be caused by:

• Certain medicines or drugs
• Infections, such as hepatitis B or C
• Other autoimmune diseases, such as lupus or rheumatoid arthritis, although these are rare  
• Genetics

What are the symptoms of vasculitis? 

Symptoms of vasculitis can vary and depend on: 

• Your type of vasculitis 
• Which parts of your body are affected 
• How inflamed your blood vessels are 

Common symptoms include: 

• Feeling tired 
• Fever 
• Muscle and joint pain 
• Feeling less hungry than normal 
• Weight loss 

Other symptoms are based on the parts of your body affected by vasculitis, such as: 

• Kidneys – blood in the urine, foamy or bubbly urine, fluid retention leading to swelling of the legs or feet, itching, metallic taste in the mouth 
• Eyes – redness of the whites of the eyes, itching, burning and changes in vision 
• Nose – sinus infections, sores or ulcers inside the nose, nosebleeds, and runny nose 
• Ears – ear infections, dizziness, ringing in the ears and hearing loss 
• Digestive tract – sores or ulcers in your mouth or stomach, diarrhea, stomach pain and throwing up blood 
• Lung – trouble breathing, bleeding in your lungs and coughing up blood 
• Nerves – numbness, tingling, feeling weak and shooting pains 
• Skin – painful rash, bruises, itching and open sores

Can vasculitis affect my kidneys?

Some types of vasculitis, called renal vasculitis, are more likely to affect your kidneys.  

The two main types of renal vasculitis are: 

• Microscopic polyangiitis (MPA)which affects small-to medium-sized blood vessels, often in your kidneys, lungs, nerves, skin and joints 
• Granulomatosis with polyangiitis (GPA)which often affects small-to medium-sized blood vessels in your kidneys, lungs and sinuses  

There is a lot of overlap in symptoms of MPA and GPA.  You cannot tell which you have based only on symptoms. 

In renal, or kidney, vasculitis, the damaged blood vessels are in the kidneys. An important part of the kidneys is the glomerulus, which is formed by tiny blood vessels called capillaries, and is responsible for filtering waste out of your blood. When these blood vessels are damaged, your kidneys cannot filter blood as well as normal. Over time, this damage can lead to kidney injury or kidney failure. 

How will I know if vasculitis is affecting my kidneys?

If vasculitis affects your kidneys, you may or may not have symptoms. Symptoms of kidney disease usually don't appear until later stages of kidney disease, when your kidneys do not work as well to filter waste and fluid out of your blood.

Your doctor can do tests to check how well your kidneys are working, such as urine (pee) and blood tests  or a biopsy, which is a procedure where doctors removes a small sample of your kidney to look at under a microscope.

How can doctors tell if I have vasculitis?

Vasculitis can be hard to diagnose and symptoms of vasculitis can vary depending on the type of vasculitis you have. Because symptoms of vasculitis may be similar to other health conditions, it can be hard for doctors to know if you have vasculitis. It may take a long time for doctors to diagnose you with vasculitis. 

Your doctor may do testsand procedures such as: 

• Blood tests to look at levels of certain blood cells and proteins in your blood 
• Urine (pee) tests to look for kidney damage 
• Imaging tests, such as X-rays, computed tomography (CT) scans, magnetic resonance imaging (MRI) and ultrasounds of your kidneys, lungs, or other organs 
• A biopsy, which is a procedure where doctors remove a small sample of an organ or tissue to look at it under a microscope. They look for specific signs of inflammation or tissue damage

How can doctors tell if I have MPA or GPA? 

To tell if you have MPA or GPA, your doctor may do a specific blood test called an ANCA test. This test looks for antineutrophil cytoplasmic auto-antibodies (ANCAs) in your blood. ANCAs are proteins that your immune system makes and that mistakenly attack your own cells. ANCAs in your blood may mean you have MPA or GPA, but it is important to note that ANCAs are not specific to only MPA and GPA, and a small percentage of those with MPA or GPA do not present with ANCA positivity.  

An ANCA test alone does not tell doctors if you have MPA or GPA. Doctors may also do some of the tests and procedures listed above. 

Doctors may not test for MPA or GPA right away if you have kidney damage. If you do not know the cause of your kidney disease, you may want to ask your doctor about testing for vasculitis. This can include an ANCA test, along with some of the other tests mentioned above.

How do doctors treat vasculitis?

Doctors treat vasculitis in different ways based on the type of vasculitis and how severe your symptoms are. The goal of treatment is to stop your immune system's attack on your own cells, to prevent damage to tissues and organs. Your doctor will work with you to make a treatment plan.  

Doctors may treat vasculitis with prescription medicines that lower the immune response in the body. 

If you have very severe vasculitis, doctors may also treat it with plasma exchange. This is a procedure in which a machine removes your plasma and replaces it with plasma from another person or with saline. Plasma is the liquid part of your blood that does not include your red and white blood cells. Plasma exchange removes antibodies that cause inflammation and injury. 

Your medical team  

Your medical team may include a variety of specialists along with your primary care doctor: 

• Rheumatologists – doctors who treat the immune system and diseases of the muscles and bones, such as arthritis 
• Nephrologists – kidney doctors  
• Pulmonologists – lung and respiratory (breathing) system doctors  
• Otolaryngologists – ear, nose and throat doctors 
• Dermatologists – skin doctors 
• Neurologists – doctors who treat the nervous system

How can I prevent vasculitis from causing further kidney damage? 

If you have vasculitis, you may help prevent it from causing further kidney damage:  

• Take your medicines as your doctor tells you  
• Go to your doctor visits  
• Get any tests your doctor recommends 
• Talk to your doctor about any new or worsening symptoms 
• Incorporate lifestyle changes, like following a healthy eating plan, being active, limiting alcohol, or quitting smoking or using tobacco. 

Where can I learn more about vasculitis?

If you or a loved one has been diagnosed with vasculitis learn more and connect with others by visiting the Vasculitis Foundation.