Granulomatosis with Polyangiitis

Also known as: GPA, Wegener’s granulomatosis

A form of ANCA-associated vasculitis that inflames small blood vessels, often in the sinuses, lungs and kidneys.

Video lesson

In the studio

Dr. Tahir is recording this lesson. Check back soon.

Animated explainer

Step 1 of 4

How it works · Vasculitis

Blood flows freely

Healthy blood vessels have smooth, flexible walls and an open channel.

What it is

GPA causes inflammation of small vessels along with clusters of immune cells called granulomas. It can damage the nose, sinuses, ears, lungs and kidneys, sometimes quickly.

Symptoms

Persistent sinus congestion, nosebleeds or crusting, ear problems, cough or coughing blood, shortness of breath, joint pain, rash, fatigue, weight loss, and blood or protein in the urine.

How it’s diagnosed

ANCA blood tests (usually PR3-ANCA) support the diagnosis. Urine tests, kidney function, chest imaging and often a biopsy confirm it and show which organs are involved.

Treatment

Rituximab or cyclophosphamide plus steroids bring the disease under control. Avacopan can reduce steroid needs. Maintenance therapy, often rituximab, prevents relapse.

Living with it

Regular urine and kidney checks catch relapses early. Vaccines and infection prevention are important while on immune-suppressing treatment.

← Polymyalgia Rheumatica

Microscopic Polyangiitis →

At a glance