As Wegener’s Granulomatosis can affect the blood vessels in many different parts of the body, the potential list of symptoms can be lengthy. The disease usually begins with an unspecific feeling of being unwell and this is what may take you to the doctor in the early stages. You may start to have flu-like symptoms; you might be very tired, lose your appetite, have a fever and have aching muscles and joints. Up to 50% of Wegener’s patients suffer with a fever and up to 35% experience weight loss.

The early symptoms include:

  • A general ill feeling
  • A persistent unexplained fever
  • Night sweats
  • Fatigue and weakness
  • Loss of appetite and weight loss
  • Joint pain and soreness

Upper airways

It is very common for Wegener’s to attack the ears, nose and sinuses.  Around 70% of all Wegener’s patients have ear, nose or throat problems at the onset of the disease and over 90% go on to develop those symptoms as the disease progresses. You may have severe headaches and pain in your face. It is likely that your nose will be blocked, you may have nosebleeds and you may get blood clots. A persistent runny nose is common as are mouth ulcers and nose ulcers.

You may experience some deafness due to inflammation of your sinuses. As well as hearing loss you may have earache, ear infections and have a discharge from your ears. Some Wegener’s sufferers have grommets inserted but these rarely alleviate deafness or discomfort. Less common problems include tinnitus, vertigo and balance issues. GPA will normally affect each ear to a different degree, and this helps distinguish Wegener’s from simple hearing loss due to ageing.

Some Wegener’s sufferers also have eye problems. Your eyes may constantly be red and painful, and you might have an increased sensitivity to light. They may be dry or gritty. Many forms of inflammation will not affect your vision, such as episcleritis, which is an inflammation of the outer coating of the eye. However, if the inflammation is of the cornea (keratitis), inside of the eye (uveitis) or the outer white part of the eye (scleritis), and it is left untreated, then your vision may be impaired. Scleritis in particular can be very painful until it is treated.

You may also get orbital inflammation in the eye socket itself. Here you may suffer from double vision, your eye may protrude (proptosis) and your eyelid may be affected, dropping lower than normal or pulling higher than usual. If the optic nerve is damaged then your vision may be blurred.

Lungs and kidneys

As well as the eyes, ears, nose and throat, the other organs most often affected are the lungs and kidneys.

Roughly half of all Wegener’s sufferers complain of respiratory problems in the early stages and this rises to nine out of ten patients in the later stages. You might develop a persistent cough and you may sometimes cough up blood. Your breathing may be noisy and hoarse and you might have a shortness of breath. You may even feel some chest pain (pleuritis). Some patients can be at risk of a blood clot forming in the lung, known as a pulmonary embolism.

Some people, perhaps a quarter of GPA sufferers, develop subglottic and tracheal stenosis. Computerised Tomography (CT) scans may show mucosal thickening below the vocal cords and the windpipe may narrow by up to 90%. This can be treated with conventional immunosuppressive treatment, mechanical dilation of the stenosis and long-acting local corticosteriods may be of therapeutic benefit. Dilation is a relatively simple procedure which can be reapeted to a very complex and serious symptom. Sometimes the narrowing is so severe that a tracheostomy is required.

The majority of Wegener’s sufferers have issues with their kidneys, if not initially then later as the disease advances. Your doctors will be checking that your urine production is normal and hasn’t reduced, and that you have no blood in your urine. As GPA could lead to organ failure and loss of renal function, checking the kidneys is a high priority.

Other organs

Some people with Wegener’s have skin ulcers, raised red or purple spots due to vasculitis (purpura), or small blisters. In rarer cases there may be some necrosis as the skin tissue dies.

Less commonly, the heart can be affected by Wegener’s. If so then the heart sac can be inflamed (pericarditis), you may have heart palpitations or angina.

If the bowels are affected then you may suffer abdominal pain, diarrhoea or bleeding.

The nervous system can be touched which can lead to muscle weakness, or soreness. You may have numbness or pins and needles in the fingers, toes or other limbs.

Image provided by Jeffrey Montes under Creative Commons licence.