Wegener's granulomatosis is a rare disease that causes inflammation of the blood vessels, which in turn limits blood flow to various organs and can lead to serious damages to the body.
Wegener’s granulomatosis generally affects blood vessels in the nose, sinuses, ears, lungs and kidneys. In more severe cases, it can affect the skin, eyes, spinal cord and heart. The condition often worsens rapidly, affecting the blood vessels and the organs they supply. It can also produce a type of inflammatory tissue called granuloma found in the blood vessels that can destroy normal tissue within an individual’s body.
Seeking medical attention is vital for a proper diagnosis and treatment of Wegener's granulomatosis. Left untreated, Wegener's granulomatosis can lead to death, mostly due to loss of kidney function.
Signs and symptoms of Wegener's granulomatosis may develop spontaneously or gradually over several months. They may include:
- Constantly runny nose, with pus-filled discharge
- Sinus pain and inflammation
- Ear infections
- Cough, with or without blood
- Chest pain
- Shortness of breath
- Unintended weight loss
- Joint aches and swelling
- Blood in urine
- Rashes or skin sores
- Eye redness, burning or pain
The exact cause of Wegener's granulomatosis remains unknown. Researchers believe that it generally develops after certain factors trigger an abnormal reaction of the immune system. The triggering factor may be an infection, though no specific infection has been recognized as the cause. In turn, the blood vessels and tissues become inflamed and restrict blood supply to various organs in the body.
Factors that can increase the risk of developing Wegener’s granulomatosis include the following:
- Age (most common between the ages of 40 to 65)
- Race (most common in individuals of Caucasian descent)
Possible complications that can arise from Wegener’s granulomatosis include the following:
- Hearing loss
- Scarring of the skin
- Coughing up blood
- Heart attack
- Kidney damage (glomerulonephritis)
- Loss of kidney function
Since Wegener's granulomatosis is a life threatening condition, treatment typically involves receiving antibiotics while regularly monitoring for any changes that might arise. A full recovery can be expected within a few months, though in some cases, prolonged antibiotic treatment may be necessary.
Treatment options may include the following:
- Corticosteroids: Medications that help suppress the immune system and treat the early signs and symptoms of Wegener's granulomatosis.
- Immune suppressant medications: Medications that help neutralize the body’s immune reaction.
- Biologic therapy: A type of therapy that involves reducing the number of a type of cell in the body (cell B) that is associated with inflammation.
Additional treatment options include the following:
- Plasmapheresis: A process in which the liquid portion of the blood (plasma) is removed and separated from the blood cells, after which the blood cells are returned to the body, where it can produce new healthy plasma to make up for what was removed. This process is also known as plasma exchange.
- Kidney transplant: A surgical procedure that involves the transfer of a healthy kidney from one individual (donor) into the body of another (recipient) who has little or no kidney function.