Granulomatosis with polyangiitis is an uncommon disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys.
Granulomatosis with polyangiitis, formerly called Wegener's granulomatosis, is one of a group of blood vessel disorders called vasculitis. It slows blood flow to some of your organs. The affected tissues may develop areas of inflammation called granulomas, which sometimes affect how these organs work.
Early diagnosis and treatment of granulomatosis with polyangiitis may lead to a full recovery. Without treatment, granulomatosis with polyangiitis can be fatal.
Signs and symptoms of granulomatosis with polyangiitis may develop suddenly or over several months. The first warning signs usually involve areas of your respiratory tract, such as your sinuses, throat or lungs. The condition of people with this disease often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys.
Signs and symptoms of granulomatosis with polyangiitis may include:
- Runny nose, stuffiness, sinus infections and nosebleeds
- Coughing, sometimes with bloody phlegm
- Shortness of breath or wheezing
- Fatigue and general aches and pains
- Numbness in your limbs, fingers or toes
- Weight loss
- Blood in urine (hematuria)
- Skin sores or bruising
- Eye redness, burning or pain
- Ear infections
For some people, the disease affects only the lungs. When the kidneys are affected, you may not notice any early warning signs. But blood and urine tests can detect the problem. Without treatment, kidney failure and anemia often occur.
When to see a doctor
See your doctor if you have a runny nose that doesn't respond to over-the-counter cold medicines, especially if it's accompanied by nosebleeds and pus-like material, coughing up blood, or other warning signs of granulomatosis with polyangiitis. Because this disease can get worse quickly, early diagnosis is key to getting effective treatment.
No one knows exactly what causes granulomatosis with polyangiitis. It appears to develop after an infection or other inflammation-causing event triggers an abnormal reaction from your immune system.
This reaction can lead to inflamed, constricted blood vessels and harmful inflammatory tissue masses (granulomas). Granulomas can destroy normal tissue, and narrowed blood vessels reduce the amount of blood and oxygen that reaches your body's tissues and organs.
Granulomatosis with polyangiitis can occur at any age. It most often affects people between the ages of 40 and 65.
Besides affecting your nose, throat and lungs, granulomatosis with polyangiitis may affect your skin, eyes, ears, kidneys, heart and other organs. Complications may include:
- Hearing loss
- Skin scarring
- Heart disease
- Kidney damage
- A loss of height in the bridge of the nose (saddling) caused by weakened cartilage
- Deep vein thrombosis