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Pauci-Immune Vasculitis

Pauci-immune vasculitis — also known as pauci-immune glomerulonephritis — is a rare condition that causes damage to the blood vessels of the kidney, leading to rapid loss of kidney function.

When your kidneys are damaged and don’t work properly, harmful substances can build up in your body. In some cases, pauci-immune vasculitis can lead to kidney failure.

The experts at UPMC specialize in treating all types of kidney disease. Our team will find the cause of your condition and develop a treatment plan to manage your symptoms and reduce your risk of complications.

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What Is Pauci-Immune Vasculitis?

Pauci-immune vasculitis — also known as pauci-immune glomerulonephritis — is a rare condition that causes damage to the kidney's small blood vessels, leading to rapid loss of kidney function.

When your kidneys are damaged and don’t work properly, harmful substances can build up in your body. In some cases, pauci-immune vasculitis can lead to kidney failure.

What are the types of pauci-immune vasculitis? 

Pauci-immune vasculitis most often occurs as part of an autoimmune condition that causes inflammation and damage to the small blood vessels throughout your body. These autoimmune conditions include:

  • Eosinophilic granulomatosis with polyangiitis (EGPA).
  • Granulomatosis with polyangiitis (GPA).
  • Microscopic polyangiitis (MPA).

Rarely, pauci-immune vasculitis can be limited to the kidney without affecting the rest of your body.

What causes pauci-immune vasculitis?

Pauci-immune vasculitis is most often caused by antineutrophil cytoplasmic antibodies (ANCA), which trigger an autoimmune response that leads to damage and inflammation in the small blood vessels that supply your kidneys.

What are pauci-immune vasculitis risk factors and complications?

Pauci-immune vasculitis risk factors

Researchers aren’t sure what triggers ANCA production and increases the risk of pauci-immune vasculitis. Potential risk factors may include:

  • Exposure to chemicals or drugs.
  • Family history of autoimmune diseases.
  • Smoking.
  • Viral or bacterial infections.

Complications of pauci-immune vasculitis

The main complication of pauci-immune vasculitis is rapidly progressing glomerulonephritis (RPGN), which can cause kidney failure.

How can I prevent pauci-immune vasculitis? 

You may be unable to prevent pauci-immune vasculitis, but taking care of your kidneys may help reduce your risk. To reduce your risk of pauci-immune vasculitis, you can:

  • Control chronic conditions — See a doctor for regular care if you have diabetes or high blood pressure.
  • Eat healthy — Eat a healthy diet with lots of fruits and vegetables. They're high in potassium, which promotes healthy blood pressure. Limiting salt from shakers and processed foods also helps reduce your blood pressure.
  • Exercise — Aim for at least 30 minutes of moderate-intensity physical activity most days of the week.
  • Get preventive care — Routinely check your blood pressure and blood sugar (glucose).
  • Keep track of medications — Inform your doctor if you routinely take over-the-counter drugs or herbal supplements and avoid or limit the use of NSAID medications (e.g., ibuprofen, naproxen).
  • Manage stress — Take steps to manage stress and get at least 7-8 hours of sleep each night.
  • Reduce alcohol intake — Limit alcohol to one drink a day for women and two drinks per day for men.
  • Stop smoking — Quit smoking if you smoke cigarettes or marijuana.
  • Treat infections quickly — Get medical care if you think you have an infection — especially strep throat or a sexually transmitted infection.

How common is pauci-immune vasculitis?

Pauci-immune vasculitis is a rare condition, affecting about 3 in 1 million people in the U.S.

What Are the Signs and Symptoms of Pauci-Immune Vasculitis?

Some people experience flu-like symptoms before developing pauci-immune vasculitis, with symptoms including:

  • Fever.
  • Joint and muscle pain.
  • Weakness or exhaustion.
  • Weight loss.

Pauci-immune vasculitis may cause symptoms of kidney failure, including:

  • Blood in your urine.
  • High blood pressure.
  • Protein in your urine.

Depending on your type of pauci-immune vasculitis, you may also experience symptoms that affect your nose, throat, lungs, nerves, skin, or musculoskeletal system.

When should I see a doctor about my pauci-immune vasculitis symptoms?

If you experience flu-like symptoms that don’t get better after a few days, sudden high blood pressure, or blood in your urine, you should schedule an appointment with your doctor right away.

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How Do You Diagnose Pauci-Immune Vasculitis?

If you have symptoms of pauci-immune vasculitis, your doctor will perform a physical exam, talk to you about your health history, and order blood and urine tests to diagnose your condition.

What to expect during your visit

During your visit, your doctor will:

  • Ask about any medications you’re taking.
  • Perform a physical exam.
  • Order blood or urine tests.
  • Review your health history.

Tests to diagnose pauci-immune vasculitis

Your doctor may order blood, urine, or other tests to check for signs of pauci-immune vasculitis.

Blood tests include:

  • Antineutrophil cytoplasmic autoantibody (ANCA) assay — Checks the levels of ANCAs in your blood.
  • Blood urea nitrogen (BUN) — Shows how well your kidneys are filtering urea nitrogen, a waste product, from your blood.
  • Estimated glomerular filtration rate (eGFR) — Shows how efficiently your kidneys are functioning based on your age, creatinine level, and sex.
  • Serum (blood) creatinine — Shows how well your kidneys are filtering creatinine, a waste product, from your blood.

Urine tests include:

  • Urine albumin-to-creatinine ratio (uACR) — Looks for high levels of a protein called albumin in your urine.
  • Urine output — Tracks how much urine your body produces each day.
  • Urine protein-to-creatinine ratio (uPCR) — Looks for high levels of all types of protein in your urine, not just albumin.

Other tests include:

  • CT scan — A test that creates images of your body's organs, bones, and tissues.
  • Kidney biopsy — A procedure that involves taking small pieces of kidney tissue for examination under a microscope to check for signs of disease. This test is used to confirm a pauci-immune vasculitis diagnosis.
  • Ultrasound — Uses sound waves to create images of your organs to check for problems.

Pauci-immune vasculitis prognosis

Pauci-immune vasculitis is a condition that is associated with rapid loss of kidney function, often within days or weeks. Although some people respond well to treatment, others may progress to kidney failure and need dialysis or a kidney transplant.

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How Do You Treat Pauci-Immune Vasculitis?

The goal of pauci-immune vasculitis treatment is to keep your condition from getting worse and prevent permanent kidney damage. Your treatment will depend on the type and severity of your symptoms.

Treatment may include:

Dietary changes

Dietary changes alone will not be enough to treat pauci-immune vasculitis, but reducing the amount of salt, fluid, and protein you eat may help your kidneys function better. Your doctor may refer you to a registered dietitian, who will recommend dietary changes to support kidney health.

Medicine to treat pauci-immune vasculitis

Depending on the cause of your pauci-immune vasculitis, your doctor may prescribe medication to:

  • Manage blood pressure.
  • Reduce inflammation.
  • Suppress your immune system.
  • Treat an infection that is causing your condition.

Plasmapheresis for pauci-immune vasculitis

If a problem with your immune system is causing your pauci-immune vasculitis, your doctor may recommend a treatment called plasmapheresis.

What happens during plasmapheresis? 

Plasmapheresis involves using a machine to separate the liquid part of your blood, called plasma, from your blood cells.

If you have an autoimmune disorder, your plasma contains antibodies that mistakenly attack healthy parts of your body. Removing plasma from your blood also removes harmful antibodies. After your plasma is separated and removed, it will be replaced with intravenous fluids or healthy donated plasma.

Why choose UPMC for pauci-immune vasculitis care?

When you choose UPMC for pauci-immune vasculitis care, you will receive:

  • Access to world-class nephrology expertise — Our world-renowned experts treat the full spectrum of kidney diseases using the latest diagnostic and treatment techniques.
  • A full range of treatment options — We'll work with you to develop a treatment plan that slows the progression of your disease as much as possible and improves your quality of life.
  • Multidisciplinary care — If you need advanced kidney treatment, we partner with special dialysis clinics, experts in supportive care, and kidney transplant surgeons.

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By UPMC Editorial Staff. Last reviewed on 2025-04-24.