Overview

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.

Source: http://www.mayoclinic.com

Symptoms

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
• Fever
• 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.

Source: http://www.mayoclinic.com

Causes

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.

Source: http://www.mayoclinic.com

Diagnosis

Your doctor will ask you about your signs and symptoms, conduct a physical exam, and take your medical history. He or she may also have you undergo diagnostic tests and procedures, such as the following:

• Blood tests. These tests can be used to look for signs of inflammation, such as a high level of C-reactive protein or a high erythrocyte sedimentation rate — commonly referred to as a sed rate. A blood test that looks for certain antibodies — the anti-neutrophil cytoplasmic antibodies test — may be helpful because these substances appear in the blood of most people who have active granulomatosis with polyangiitis.

  Another blood test can check for anemia, which is common in people with this disease. A blood test for creatinine can check whether your kidneys are properly filtering waste products from your blood.

• Urine tests. These tests may reveal whether your urine contains red blood cells or has too much protein, which may indicate the disease is affecting your kidneys.
• Chest imaging. Imaging tests can help determine what blood vessels and organs are affected. They can also help your doctor monitor whether you're responding to treatment. You may be asked to undergo chest X-rays, computerized tomography (CT) or magnetic resonance imaging (MRI).
• Biopsy. This is a surgical procedure in which your doctor removes a small sample of tissue from the affected area of your body. It's the only way to confirm a diagnosis of granulomatosis with polyangiitis.

Source: http://www.mayoclinic.com

Complications

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

Coping and support

With treatment you're likely to recover from granulomatosis with polyangiitis. Even so you may feel stress about possible relapse or damage the disease may cause. Here are some suggestions for coping with the disease:

• Understand your condition. The more you know about granulomatosis with polyangiitis, the better prepared you may be to deal with complications, the side effects of your medications and relapses. Besides talking with your doctor, you may want to talk with a counselor or a medical social worker.
• Maintain a strong support system. Family and friends can help you cope. And you may find it informative and comforting to talk with other people who are living with the condition. Ask a member of your health care team about connecting with a support group.

Source: http://www.mayoclinic.com

Risk factors

Granulomatosis with polyangiitis can occur at any age. It most often affects people between the ages of 40 and 65.

Source: http://www.mayoclinic.com