Wegener’s granulomatosis is a type of vasculitis, or disorder that produces inflammation in the blood vessels. Wegener’s granulomatosis damages and inflames blood vessels in several organs, including the lungs, sinuses, and kidneys. Inflammation restricts blood flow and prevents oxygen from traveling to your tissues and organs, which ultimately impacts how well they can function. Granulomas, or inflamed tissue lumps, grow around blood vessels and damage organs.
Wegener’s granulomatosis is an autoimmune disease, which means that the body’s immune system turns against its own healthy tissues. This condition causes the immune system to attack blood vessels, specifically.
Experts do not know what causes Wegener’s granulomatosis. Genetics do not play a role, as Wegener’s granulomatosis does not usually run in families. Infections might cause this condition. Bacteria and viruses cause the body to send out cells that can create inflammation. That response could harm otherwise healthy tissues. However, Wegener’s granulomatosis is not connected to any fungus, bacteria, or virus.
Early stages of Wegener’s granulomatosis do not usually present symptoms. In more advanced stages, this condition targets the lungs, sinuses, and kidneys. This condition can progress quickly once symptoms appear.
Symptoms of Wegener’s granulomatosis can include:
The cause of Wegener’s granulomatosis is unknown. As such, it is difficult to identify risk factors. You can develop Wegener’s granulomatosis at any age, but the condition most often occurs between the ages of 40 and 65.
First, you will discuss your symptoms and medical history with your doctor. Then, your doctor will determine which tests to perform in order to make a diagnosis.
Urine and Blood Tests
Imaging Tests
Biopsy
Your doctor must use a biopsy to make a Wegener’s granulomatosis diagnosis. A biopsy is a surgical procedure in which a doctor will take a small tissue sample from an affected area, like your kidney or lung, and give it to a lab for diagnostic confirmation. At the lab, a technician will determine whether the tissue sample contains evidence of Wegener’s granulomatosis. If urine, blood, and imaging test results are inconclusive or abnormal, then your doctor might order a biopsy to help make a diagnosis.
Your doctor might recommend a few different strategies to treat Wegener’s granulomatosis. You might take certain medications indefinitely to keep the disease from returning. The two most common treatments for Wegener’s granulomatosis are medications and plasma exchange.
Medications
Sometimes, doctors will recommend combining drugs to reduce inflammation. A common combination would be cyclophosphamide and prednisone. Many people with Wegener’s granulomatosis recover using this medication combination.
Mild cases of Wegener’s granulomatosis might resolve using methotrexate and prednisone. Methotrexate is an immunosuppressant and prednisone is a corticosteroid. This combination has fewer side effects than cyclophosphamide and prednisone.
Side effects from these drugs can include reducing your body’s ability to fight infection and weakening your bones. Some medications also cause weight gain. Your doctor will keep tabs on whether these side effects develop and help you address them effectively.
If the Wegener’s granulomatosis is targeting your lungs, a doctor might have you take a combination antibiotic to reduce your chances of infection.
Plasma Exchange
Plasma exchange, also called plasmapheresis, takes out the liquid part of your blood, called plasma, that holds disease-producing substances. In the same treatment, you will receive either fresh plasma or a protein, albumin, that helps your body create new plasma. Your doctor will prescribe a plasma exchange if you have a severe case of Wegener’s granulomatosis in order to help your kidneys recover.
Wegener’s granulomatosis is a type of vasculitis. People living with any type of vasculitis can make some lifestyle changes that will help their body recover more easily.
See a doctor right away if you believe you have Wegener’s granulomatosis. Because this condition can present in so many different ways, you should be on the look-out for abnormal symptoms and make a doctor’s appointment if any arise.
You should see a doctor if you exhibit the following symptoms:
Wegener’s granulomatosis gets progressively worse, so it is imperative that you seek treatment right away. A treatment plan and a dedicated care team can help you resolve symptoms.
If you are living with Wegener’s granulomatosis, the outlook depends on the severity of your case and which organs it has affected. Medication can usually treat this disease, but relapses are incredibly common. You will work with your doctor on follow-up visits to make sure the Wegener’s granulomatosis does not return and cause further health complications. To prevent a relapse, you will need to take medication long-term.
Undiagnosed or untreated Wegener’s granulomatosis can progress quickly and produce health complications. Possible complications include:
Although most people with Wegener’s granulomatosis recover with treatment, you might still have some stress about your condition. You could also feel stressed about relapses and consequent health complications. To cope with your condition, you should: