Wegener’s granulomatosis

Wegener's Granulomatosis, Granulomatosis with polyangiitis

Overview

Type of disease: Rare Condition or Disease

Granulomatosis with polyangiitis (GPA), or Wegener’s granulomatosis (WG), is a rare condition that causes inflammation in the blood vessels. In addition to inflammation, GPA also causes the development of abnormal tissue, called granulomas, which can destroy normal tissue. This combination of inflamed blood vessels and destruction of normal tissue causes poor blood flow to organs, leading to permanent organ damage. The upper respiratory system, lungs, and kidneys are the areas most commonly affected by GPA. The first symptoms of this condition can include nosebleeds, runny nose with pus-filled discharge, ear infections, chest pain, shortness of breath and coughing up blood. Other symptoms may include unintended weight loss, blood in the urine, skin sores, eye redness, or fever.

The cause of GPA is unknown but researchers believe GPA develops after an initial inflammation-causing event leads to an abnormal immune system reaction. GPA can affect anyone, but most people get GPA between the ages of 45 and 80.

In addition to taking a detailed medical history and performing a physical exam, your doctor may conduct several tests to diagnose GPA. Blood tests may be used to check for a specific protein (autoantibody) that can be seen in people with GPA, called anti-neutrophil cytoplasmic autoantibodies (ANCA). Although many people with GPA have ANCAs in their blood, not everyone with GPA does. Also, ANCAs may be present in people who don’t have GPA. In addition to blood tests, your doctor may also perform urine tests (to check kidney function), chest x-rays, or they may remove and examine a small piece of tissue from an affected organ (biopsy). Treatment of GPA usually includes medications to slow down your immune system. Severe forms of GPA may require surgery. The earlier GPA is detected and treated, the more likely a person is to recover. Talk with your doctor about the most current treatment options. Support groups may also be helpful.

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