Wegener's Granulomatosis

How many know what this is? How many of you can even pronounce it? I can't. And yet, I have lost my dear friend, Elsie Harris, to this disease that seemed to come out of no where. Until Elsie's diagnosis, I had never heard of it or it's deadly affects and she would be the first to tell me to alert the public of this rare disease.

Another friend, W. Thomas Kelley, Jr., diagnosed with Wegener's Granulomatosis last year, is slowly improving. Due to early diagnosis, Tom Kelley was able to request this article himself.

It is pronounced VEG-uh-nurz gran-u-loe-muh-TOE-sis. Actually, no, that didn't help me much either.

However, the definition is just as widespread as the name is long and difficult.

Wegener's Granulomatosis is quite uncommon, only found in one out of every twenty-five thousand patients. It usually strikes between the ages of forty and sixty but can be found in patients as old as seventy and eighty. It seldom affects children and the very young, though has been seen in infants as young as three months. In rare cases, it has been found in patients in their 30s. Therefore, it cannot be said to totally illiminate anyone.

Showing no distinction, it can be found in both males and females equally.

The organs affected by Wegener's Granulomatosis are the heart, lungs, kidneys, sinuses, and others less vital . As you can see, it may affect and shut down vital organs that sustain life.

Elsie's began in her lungs and quickly spread to other organs in her body, eventually causing a full shutdown.

Below, I will cover results, symptoms, areas most commonly affected in the early stages, diagnostic tests needed, treatment available, risks, side affects, and prognosis, or hope of recovery, for those afflicted with Wegener's Granulomatosis.

The disorder causes inflammation of blood vessels, affecting the upper respiratory tract (sinuses, nose, and trachea). This, in turn, restricts blood flow to the organs of the body. The patient's eyes may also be affected ranging from mild conjunctivitis to severe swelling of the eye.

An inflammatory tissue or granuloma, which destroys normal tissue can be found in the blood vessels. It is thought to be an autoimmune disorder, though there is no known cause.

Early diagnosis and treatment is important to recovery. Unfortunately, Elsie's went undetected for some time and had caused a great deal of damage. However, Thomas' was detected early on and he had several years of life before succumbing to the disease.

Without treatment, the disease can be fatal, usually due to kidney failure.

 Symptoms of Wegener's Granulomatosis and their severity vary among patients. Frequent sinusitis is the most common symptom. Other symptoms may include loss of appetite and weight loss, fever, and fatigue.

However, most patients notice symptoms of the respiratory tract first. These include persistent runny nose (nasal crusts and sores), nasal and face pain, nose bleeds or discharge, cough with possible bloody phlegm due to lung inflammation, voice change, shortness of breath and wheezing, middle ear pain or hearing loss, eye inflammation, with or without loss of vision, joint and muscle pain, skin rashes, and kidney inflammation.

Wegener's Granulomatosis has symptoms similar to a number of other disorders, which may make it difficult to diagnose.

Many times, it is more in the form of a viral infection.

Since Wegener's Granulomatosis is a combination of symptoms, the physical examinations may involve several factors. Sometimes a biopsy of tissue from the skin, nose, sinus, lungs, or kidneys may be necessary. It can also affect the eyes. These together help to prove the diagnosis of Wegener's Granulomatosis as well as help to judge whether the disease is active or in remission.

A positive blood test for antineutrophil cytoplasmic antibodies (ANCA) can support this diagnosis, though a blood test alone cannot prove the diagnosis of Wegener's Granulomatosis. Therefore, the doctor may also order a red blood cell count, checking for anemia. Also, a sedimentation rate which shows the speed in which the blood cells settle in a vertical glass tube, a urinalysis, and chest and sinus x-rays.

Since the lungs may be abnormal even though there are no symptoms, it is important, if Wegener's Granulomatosis is suspected, to have imaging tests such as a conventional X-ray and CT scan to totally rule out the effects of the disease.

The Trachea is a characteristic respiratory tract complication of Wegener's Granulomatosis. There is a narrowing of the "windpipe" just below the vocal cords, a condition called "subglottic stenosis". This narrowing, caused by inflammation and scarring, causes difficulty breathing and may render an emergency tracheotomy necessary.

Because Wegener's Granulomatosis is often a life-threatening disease, it is treated with a variety of powerful, life-saving drugs. Treatment usually includes corticosteroid medicines, such as prednisone. Other drugs that also suppress immune function are cyclophosphamide, methotrexate, azathioprine, or mycophenolate mofetil. Such drugs usually induce remission (the complete absence of all signs of the disease).

When Wegener's Granulomatosis is in remission, the dosage of prednisone is reduced or often completely stopped. Some patients do require a low dose of prednisone to sustain remission though the ideal duration of treatment with the other immunosuppressive medications is uncertain.

However, it must be borne in mind that these therapies are not cures. It is well known that Wegener's Granulomatosis is associated with relapse. The statistics run as high as sixty percent in the first year and eighty percent by the end of the second year, once treatment is stopped.

The medicines used to treat Wegener's Granulomatosis can have serious side effects. The dosage of medicine must be adjusted as needed throughout the treatment and the patients must be monitored closely by their doctor throughout the course of treatment.

Thank you for writing this informative article. Hopefully a lot of folks will see it and benefit from it. The road to some recovery is long and bumpy. Doctor's told me that it will take 2 or 3 years to see any significant improvement, if at all. One treatment not mentioned here, and what apparently saved my life, is "Plasma Pheresis". That removes toxins, or autoantibodies that attack and destroy one's own body tissue by exchanging one's own plaza for fresh, frozen plaza. Done a total of 6 times, it is the coldest, most painful procedure that I have ever had done!! There were many other treatments as well. I was in the hospital for 11 weeks (4 in intensive care). I did have dialysis a number of times, but the kidneys are barely functional now without that. I have oxygen, @3 L/M- 24-7, I take a multitude of powerful, possibly deadly drugs everyday. I am sorry that you lost a good friend to this disease, but consider myself a lucky man. The doctor's told me that I was 2 days away from being a daisy pusher before the first plazapheresis treatment.

{Photo of Tom before his illness.}

The Inflammation of the kidneys is called Glomerulonephritis. If not treated aggressively, Wegener's involvement of the kidneys can lead to kidney failure. Renal masses can occur, but are very unusual in this disease. In a urinalysis of a patient with kidney inflammation, when Wegener's is active, red blood cells will form a clump.

These risks of side effects have stimulated research to discover other, less risky, medications. Since side effects detected during treatment aid in these discoveries, regular monitoring checks are important for measuring medication tolerances as well.

Wegener's Granulomatosis is very serious and treatment carries significant risks of its own. However, treatment is life-saving for almost everyone, when the diagnosis is made early and appropriate treatment administered.

For many with Wegener's Granulomatosis, long term survival has been accomplished, allowing them to lead relatively normal lives.

If the past preludes to the future, research will lead to further discovery and better treatment.

With this, someday, we may see a cure for the disease which robbed Elsie of her health and, eventually, her life.

Because of this research, Tom Kelley was able to return to his family for a couple of years. Unfortunately, Tom recently lost his fight with his illness...he is truly missed!

Wherever you found one, you found the other until the day Miler lost his Elsie to this debilitating disease. We all share in his grief and in his loss...