Is Wegener’s Granulomatosis an Autoimmune Disorder?

Overview of Wegener’s Granulomatosis

Granulomatosis with polyangiitis is a rare illness that causes inflammation of the blood vessels in your sinuses, nose, lungs, throat and kidneys. Previously called Wegener’s granulomatosis, the affected tissues can develop areas of inflammation called granulomas, which can affect how these organs work.

The history of Wegener’s granulomatosis dates back to 1897 when it was mentioned in an article written by Peter McBride. A German medical student, Heinz Klinger, later added anatomical information to the article. Friedrich Wegener, a German pathologist, was the one who presented the entire clinical details  of the disorder in 1936. Hence, the condition is named after him as Wegener’s granulomatosis.

What is Wegener’s granulomatosis?

Wegener’s granulomatosis  causes systemic inflammation of blood vessels. It is an extremely rare medical condition that leads to quick inflammation of blood vessels across your body. However, the most commonly  inflamed parts include the sinuses, lungs, nose, kidneys, and throat.

What are the symptoms of Wegener’s granulomatosis?

You may notice the signs of Wegener’s granulomatosis suddenly or with time. Hence, the signs and symptoms of the disorder may vary largely depending on the severity. They may also vary depending on the organ that Wegener’s granulomatosis has first targeted. Some of the symptoms of the disorder include:

• Loss of appetite causing weight loss
• Constantly running nose (rhinorrhea), nosebleeds, and pus-like mucus coming from the nose
• Frequent coughing (sometimes with blood)
• Fatigue
• Shortness of breath
• Fever
• Joint pain
• Blood in urine
• Vision impairment, ear inflammation, and skin sores

When to see a doctor for Wegener’s granulomatosis

The early signs of the disorder are similar to common flu; hence, it can become challenging to differentiate between them. But, you need to see a doctor as soon as possible if the symptoms continue even after taking medicines for flu, especially if you experience nosebleeds. Since Wegener’s granulomatosis can worsen within a short period of time, it is also advised to quickly visit a doctor for any internal pain and discomfort around the lungs, kidneys, nose, and throat. Early diagnosis will lead to better outcomes . You can head to Apollo Hospitals  straight away or request an appointment.

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What causes Wegener’s granulomatosis?

There is no clear indication of what causes the disorder. It is not a form of cancer and is not hereditary. Also, the condition is not contagious. It just appears  out of nowhere, and you start experiencing some of the symptoms, which can again be either sudden or over a few months. Wegener’s granulomatosis leads to inflammation and narrowing of blood vessels, making it difficult for the blood and oxygen to reach all the organs.

How is Wegener’s granulomatosis treated ?

Wegener’s granulomatosis is a condition that can recur. It has both short-term and long-term remissions, i.e. reduction in signs and symptoms. If the signs disappear slowly, the treatment can take longer than expected. If the remissions are quicker , you can easily lead a normal life within a few months. However, you might have to visit your doctor regularly for constant medical checks to prevent a recurrence. Regardless of the severity of your condition, the early treatment of the disorder includes  medication with immunosuppressive drugs and plasma exchange.

Medications

Wegener’s granulomatosis is an autoimmune disorder; hence, the medications for treating this disorder include drugs that can suppress your immune system. Using these drugs may have some side effects, including infections and weight gain. Hence, your doctor might prescribe other medications to reduce side effects. Some of the common immune system suppressing drugs include:

• Corticosteroids
• Cyclophosphamide
• Azathioprine
• Mycophenolate
• Methotrexate
• Rituximab

Plasma exchange

Plasma exchange, aka plasmapheresis, removes the infected plasma from your blood. Fresh plasma or albumin (a protein made by the liver) that allows your body to produce new plasma is given. In those who have very serious granulomatosis with polyangiitis, plasmapheresis can help the kidneys recover.

What are the complications of Wegener’s granulomatosis?

If the condition is overlooked or the treatment is delayed, it can lead to several fatal and non-fatal complications. Some of these complications include:

• Skin scarring
• Weakened cartilage causing shortening of the bridge of the nose
• Hearing loss
• A blood clot in the deep veins
• Kidney damage
• Lung failure

Another  problem that can lead to complications is in the treatment itself. Since Wegener’s granulomatosis is an autoimmune disease, its medication includes some drugs that suppress your immune system. This can lead to health conditions caused due to a lack of immunity.

Conclusion

Although Wegener’s granulomatosis is treatable , it can lead to several complications. Hence, it is best to visit a doctor as soon as you experience any of the above symptoms . You can visit any of Apollo Hospitals  for the best medical services and treatment. If you or any of your family members face this disorder, it is also advisable to visit a consultant for a deep understanding of the disease. This will help you cope  with the disorder and recover quickly.

Frequently Asked Questions (FAQs)

1. What are the risk factors for  Wegener’s granulomatosis?

There are no particular risk factors for Wegener’s granulomatosis. Anyone, irrespective of their sex or age, can be affected  by the disorder. Also, there is no clinical evidence to support that the disease is hereditary  .

2. What doctors will treat Wegener’s granulomatosis?

Both the doctors and treatment for the disorder rely heavily on what organ of your body is infected. For instance, a nephrologist will treat you for kidney inflammation, and a pulmonologist will treat you for lung inflammation. However, if the disorder has infected multiple organs, a group of doctors specializing in different areas may treat you.

3. How long does it take to treat Wegener’s granulomatosis?

Usually, it takes around 3 to 4 months to treat  Wegener’s granulomatosis. However, the course of treatment may vary depending on short-term and long-term remissions. Short-term remissions reduce the course of treatment.

4. What are the common diagnostic tests for Wegener’s granulomatosis?

The common tests used for diagnosing Wegener’s granulomatosis include imaging tests like X-ray, CT scan, or MRI to determine narrowed blood vessels and affected  organs. Blood tests are also useful to detect signs of inflammation. For instance, a high presence of C-reactive protein can indicate the presence of inflammation. An advanced diagnosis is made with a biopsy when a sample of the affected  tissue is taken and tested to confirm the disorder.

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