Wegener’s Granulomatosis–A Rare Inflammation in Blood Vessels that Can Lead to Death (As Seen on House MD)

This is part of our House MD Project series.

Click here to read the connection to the episode (spoiler alert!)

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Photo by NBCUniversal

Danger level: High

What is it?

Wegener’s Granulomatosis (WG for short) is an inflammation of blood vessels, restricting blood flow to various organs in the body.

Who gets it?

WG is quite rare. In the United States, for example, it’s estimated that about 3 out of every 100,000 people are sick with it. It’s more common in people of Northern European origins, and is less common in black people. It can occur in any age, but is most common in middle age (the average age when it appears is around 40). Men and women are affected equally.

What causes it?

In WG there is an inflammation of blood vessels in the body, meaning it’s a vasculitis (the medical term for inflammation of blood vessels. There are other diseases in this group – such as temporal arteritis and kawasaki, which we covered before).

Our blood vessels generally come in 3 sizes, much like clothing. Small, medium and large. WG is an inflammation of small and medium sized blood vessels.

It happens because the body actually attacks its own blood vessels. The process isn’t completely understood, but probably involves a change in the body’s immune system which triggers the body destroying its own blood vessels.

WG tends to affect mostly 3 systems in the body: The upper airways, the kidney, and the lungs. They are affected since the arteries that supply them with blood are damaged in the disease.

How does it feel?

There are some general symptoms in WG which include loss of appetite, fever, fatigue, and even weight loss.

Usually the first specific symptoms involve the upper airways and can include nosebleeds, nasal congestion, collapse of the bridge of the nose (causing a deformity called saddle nose, seen in this picture), a hole in the nasal septum (which separates our right side of the nose from the left), inflammation of the sinuses (sinusitis), ear infections, difficulty breathing, and coughing (sometimes a bloody cough).

When the disease is in the lungs people may feel shortness of breath (which may be caused by bleeding in the lungs), or cough. When the kidneys are involved, kidney failure can occur, which may be life threatening.

Other organs may be involved, such as the skin (a rash may appear, like the one on House), the joints – which can become inflamed as well, and the nerves (something which can also affect vision – like the patient on House told that had happened to her).

Believe it or not, but there are even more possible symptoms, into which we won’t get here (including, rarely, pancreatitis, which for some reason was a main manifestation on House).

How is it discovered?

The disease resembles many other diseases, making the diagnosis difficult. A blood test checking for a component in the body called ANCA can be used, since it’s elevated in the disease. A physical exam, x-ray, and urine test, among others, can help with the diagnosis. The definitive way is performing a biopsy (taking a piece of an organ to look at under a microscope) on one of the involved organs, which will show something like this:

wegener-histology

WG under a microscope. The round area on the left is called a granuloma, which is a mass of immune cells (that’s how the disease got its name). Photo by Nephron.

How is it treated?

Drugs that suppress the immune system (which attacks the body here) are used in WG. This includes steroids and chemotherapy.

What happens after treatment?

With treatment given on time, symptoms may disappear altogether. Still, in about half of patients, the disease may return. Before the disease was understood and the right treatments were given, about half of all patients died within 5 months. Today more than 75% get to live longer.


The bottom line – How do I avoid it?

Unfortunately, there is no known way to prevent WG.

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