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This is part two of the blog entry entitled: Two serious headaches that can mimic sinus disorders.
The first was Temporal arteritis. The second is: read on . . . .
A very small number of people with severe chronic headaches and nasal or sinus complaints will suffer from the condition called wegeners disease. It is important to note that this is an extremely rare disease, affecting about one in 25,000 people. The symptoms of wegeners disease are due to an inflammatory process that affects the blood vessels and other tissues of the body. It is considered an immunologic disease, but the cause of wegeners disease is unknown. There’s no evidence that it’s contagious or hereditary.
Wegeners patients may have symptoms of severe headache associated with more unusual symptoms such as migrating joint pains, weight loss, drenching night sweats, nose bleeds, or breathing problems. Even if you checked off the symptoms, the odds are low that you have wegeners disease, because this is such an unusual condition. However, as you read on, if you feel that your symptoms are suspicious for this disease, you should certainly discuss the possibilities with your health care provider.
Wegeners disease can come on slowly over a period of weeks to months, sometimes with subtle symptoms that gradually become more problematic. The disease can also develop rapidly with severe symptoms. It usually develops in a patients 30s or 40s, but occasionally presents an earlier age or an overage. It affects Caucasians more than other ethnic groups.
If you’re reading this text, you are probably bothered by headaches or nasal complaints, which are quite common in patients with wegeners disease. About 80 to 90% of patients will develop nasal symptoms which include nasal pain, nasal bleeding, bloody or dry mucus crusts, a foul odor from the nose, nasal obstruction, and sinus pain. Occasionally patients with nasal complaints also have numbness or pain of the front two upper Central incisor teeth, because these teeth share in nerves in common with the nasal cavity.
Knowledge of the anatomy of the nose and sinuses helps one understand the other symptoms that can occur with wegeners disease. If the disease extends back to the post here nasal cavity or nasopharynx, it can affect the eustachian tubes and cause fluid in the years are hearing loss. Extension into the sinuses can cause sinus pain, inflammation, or bacterial infections. If the wegeners disease extends into the orbit, it can cause redness of the lesions, swelling, pain, limited motion of the eye, double vision, and occasionally blindness. If the disease process extends into the brain, it can cause meningitis, neurologic symptoms, seizures, stroke, neurologic deficits, meningitis, or inflammation of the brain, called encephalitis. However, it is more common for wegeners disease to remain within the nasal cavity and/or sinuses than to extend elsewhere.
Involvement of the lower respiratory tract also occurs in patients with wegeners disease. There are two particular areas of involvement of the lower respiratory tract (that is below the vocal cords) that are important to recognize. First, Wegeners disease can affect the “subglottis”, which is the airway just below the vocal cord or glottis. The subglottis can be thought of as the very first part of the trachea, which connects the throat and voice box with the lungs. Wegeners disease causes inflammation of the tissues in the subglottis and will cause an airing of the airway, so patients will complain of difficulty breathing, particularly when they exert themselves. Although this may be difficult to distinguish from other lung diseases or heart diseases, there is one feature of narrating in the subglottis that is apparent and readily detectable if a person is considering it. That is, patients with subglottic narrowing (also called subglottic stenosis) will complain of noisy breathing on inhalation as well as exhalation. If a patient is asked where they feel the obstruction is occurring, many of them will .directly toward their voice box, or at the subglottis, just below the voice box. As the narrowing of the subglottis becomes worse, patients will find their exercise tolerance, even for walking, may decrease from 200 yards, to 100 yards common to 50 yards, to 25 yards or less before they are short of breath and breathing noisily as well as heavily. This noisy breathing is called Strider, and when it occurs on inspiration and expiration it is called biphasic stridor. It is different than the noisy breathing of asthma. The noisy breathing in asthma asthma typically occurs on expiration far more than inspiration.
The lower respiratory tract involvement of wegeners disease can also occur in the lungs, where symptoms of lung involvement can mimic pneumonias. Patients can have shortness of breath, chest pain, cough, fever’s, and can have any type of x-ray abnormality of the lungs. A radiologist may have a difficult time distinguishing x-ray findings of wegeners disease from other lung diseases such as pneumonia, but when the x-ray findings are taken with the patient’s complaint and other physical exam findings, the diagnosis is usually more apparent to a radiologist.
One of the other hallmark findings of wegeners disease is involvement of the kidneys. Not every patient with wegeners disease has kidney involvement. In fact, there are many different combinations of findings that can occur. Some patients have very “focal wegeners disease” with involvement of only the nose, or only the subglottis, or only the lungs. Other patients have the “full-blown” form of wegeners disease and may have nose, sinus, subglottic, lung, and kidney disease. Kidney disease is often relatively asymptomatic. The inflammation and vasculitis it occurs in the kidney will often cause microscopic amounts of blood to leak into the urine, and this microscopic blood may show up on a urine analysis. As the disease progresses, the functioning of the kidney can be compromised, but is typically is associated with relatively few symptoms.
Although involvement of the nose, sinuses, subglottis, lungs, and kidney are some of the typical areas of involvement, it is not unusual for a patient with wegeners disease to have other symptoms and signs. Because some of these symptoms affect the whole body, they are called ” constitutional symptoms”. The symptoms include unexplained fevers, drenching night sweats (soaking pajamas or bedsheets), chills, unexplained significant weight loss, significant fatigue. When I see patients with a new diagnosis of wegeners disease, I find that the drenching night sweats, when they occur, are helpful to making the diagnosis. The other symptoms that is remarkable in patients with wegeners disease (is when it occurs) is the joint pains or migrating joint pains. Patients who were otherwise very healthy will complain of severe joint pains, often migrating from area to area. These will become very problematic and in some cases debilitating, to the extent that a patient cannot walk without severe pain, climb stairs easily, pick up a gallon of milk, or bend their elbow without significant pain.
If you and your doctor are concerned about wegeners disease, the workup typically requires blood tests, chest x-rays, a urine test, kidney tests, and an ENT consultation, pulmonary consultation, or rheumatologist consultation. A particular blood tests, called a ANCA, will typically be ordered, along with other tasks for signs of inflammation such as an ESR or CRP. If a urine examination to look for red blood cells or other signs of kidney disease is helpful to rule out kidney involvement and a blood test for kidney function called a BUN and creatinine is helpful. A chest x-ray is often warranted to look for symptomatic or non-symptomatic lung lesions, particularly in patients that have the constitutional symptoms noted above or any chest or lung complaints.
Treatment of wegeners disease involves immunosuppressive drugs to stop the inflammation of the vessels, that is treat the vasculitis. After the disease is in remission, the immunosuppressants can be tapered to a lower level in order to maintain a patient in remission and prevent further damage to the tissues that have been involved in any one particular patient.
There are a number of online resources for patients with wegeners disease, the most thorough of which is the vasculitis foundation website: http://www.vasculitisfoundation.org/wegenersgranulomatosis . This site contains excellent information on the disease. It’s a bit scary to read some of the factual material on the disease. I recommend patients read it only after they are certain they have the disease, and read it after having some discussion with a knowledgeable specialist, so any questions or concerns one might have can be answered by a caregiver with whom one already has a relationship. Remember, this is a rare disease.
Jeffrey E. Terrell, MD