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Patient education: Granulomatosis with polyangiitis (The Basics)

Patient education: Granulomatosis with polyangiitis (The Basics)

What is granulomatosis with polyangiitis? — This is a rare disease that causes pain and swelling of blood vessels and other tissues. It can affect organs or structures anywhere in the body. In most cases, granulomatosis with polyangiitis affects the nose, mouth, ears, lungs, and kidneys.

What are the symptoms of granulomatosis with polyangiitis? — The symptoms include:

Runny nose – The discharge from the nose can include pus or blood and does not go away.

Small sores in the nose or mouth

Sinus infections – The sinuses are hollow areas in the bones of the face.

Ear problems, including fluid leaking from the ear, ear pain, ear infections, and hearing loss

Inflammation and breakdown of "cartilage" – Cartilage is the tough, elastic tissue found in the ears, the windpipe, and the spaces between bones.

Hoarse voice

Cough

Wheezing and breathing problems

Chest pain

Blood in the urine

Red or purple spots on the skin, most often on the lower legs

Is there a test for granulomatosis with polyangiitis? — Yes. Your doctor or nurse will ask about your symptoms and do an exam. Other tests include:

Blood tests

Urine tests

X-ray or CT scan of the chest – These are imaging tests that create pictures of the inside of the body.

Biopsy – The doctor takes a small sample of tissue, usually from the skin or the kidney. Sometimes, the sample is taken from inside the nose or the lungs. Then, another doctor examines the sample in a lab.

How is granulomatosis with polyangiitis treated? — The treatment happens in 2 parts, 1 after the other. It involves medicines that suppress the body's infection-fighting system, called the "immune system."

The first part of treatment is called "induction." You will need to take strong medicines that are given as pills or through a thin tube that goes into a vein, called an "IV." You will need to see your doctor at least once a month during this part of treatment. That's because the medicines can have serious side effects. This part lasts until your symptoms get better, which usually takes about 6 months.

The second part of treatment is called "maintenance." For this part, your doctor will switch you to a lower dose of the medicines. Or you might take different medicines that have less serious side effects. The maintenance part lasts at least 1 year.

More on this topic

Patient education: Chronic kidney disease (The Basics)
Patient education: Kidney biopsy (The Basics)

Patient education: Chronic kidney disease (Beyond the Basics)
Patient education: Kidney (renal) biopsy (Beyond the Basics)

This topic retrieved from UpToDate on: Jun 02, 2024.
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