Without treatment Wegener's sufferers could die within a few months. Before effective therapies were discovered the mean survival of adults with GPA was just five months. 82% of patients died within the first year and 90% within the second year. With the introduction of corticosteroids as a treatment that mean life expectancy rose to twelve months. It was only when prednisone and cyclophosmamide were used in conjunction did the remission rate rise to over 90%. With appropriate treatment the outlook for Wegener's Disease sufferers is good.
The prognosis in Wegener's Disease is dependant on how severely the disease has attacked the sufferer and the damage that was done to the major organs such as the lungs and especially the kidneys in the active phase of the disease. Wegener's Disease may be present for months or even years before it is diagnosed, and it can often initially be misdiagnosed as another disease altogether.
An early diagnosis of Wegener's Granulomatosis means a better outlook. About half of all Wegener's sufferers will improve with treatment but then go on to relapse (have a 'flare') within two years of stopping their treatment. You may have many such flares in the years following your diagnosis which will require a temporary increase in your treatment. This treatment will then be reduced as you go into 'remission.'
If the Wegener's is not caught early then the inflammation may affect the lungs and kidneys. This could potentially lead to lung damage or kidney failure. Your eyes could also be affected as well as your nose. Many people suffer from nasal septum perforation. If you present with this symptom, your doctor may suspect that you are a heavy drug user and that the hole in your nose may have been caused by snorting illegal drugs!
Long term damage
You may have to learn to live with permanent damage such as end-stage renal disease, chronic pulmonary dysfunction, hearing loss, destructive sinus disease, saddle nose deformities, perforation of the nasal septum, proptosis or even blindness.
Respiratory problems may occur following an upper-airway obstruction such as subglottic stenosis, or pulmonary involvement such as pleural effusion, dyspnea, diffuse alveolar haemorrhage. If your airways narrow you may need regular dilation every five or six months.
Many other long term health problems can occur as a result of the treatment of Wegener's, such as osteoporosis or bladder cancer. Some studies have shown that Wegener's sufferer's have up to twice the risk of developing cancers such as leukaemia, lymphoma, and nonmelanoma skin cancers.
One in seven Wegener's patients experienced a cardiovascular event within five years of diagnosis. This is over 3 times the expected number in those without Wegener's Disease.
If you have suffered hearing loss as a result of Wegener's Disease this may return after treatment as the excess liquid in the inner ear regresses. However, if the damage to the inner ear is severe, the hearing loss may be permanent.
The overall ten year survival rate for Wegener's Disease is between 75% and 88%. Poorer survival rates are linked to old age and whether organ damage has occurred. For those with an absence of kidney involvement the five year survival rate is 100%. If the Wegener's sufferer has renal disease then the five year survival rate drops to 70%.
The most common causes of death in Wegener's Granulomatosis include infections, respiratory and renal failures, malignancies, and cardiovascular events.
The outlook for people with Wegener's Disease is undoubtedly far better today than it was a generation or two ago. Those with GPA can lead relatively normal lives. You may need to take medicine regularly and you will be monitored by doctors every few months to check for signs of a relapse. With new drugs such as Rituximab and ongoing research I am hopeful that the prognosis for Wegener's patients will improve even further in the years to follow.
Image provided through Creative Commons Licence by Hartwig HKD.