Granulomatosis with polyangitis used to be called Wegener’s disease and sometimes doctors still call it that. Most call it GPA for short. No matter what it’s called the results are the same. The blood vessels are inflamed and weakened by this rare disease. Blood vessels to vital organs such as the lungs, kidneys and the sinuses are most often harmed but blood vessels anywhere in the body may be harmed.
The cause of this potentially deadly disease is unknown but it is not contagious and not a genetic condition. The good news is that patients with GPA live an average of eight more years if they get treatment early enough.
Symptoms vary depending what particular blood vessels are affected.
The most common symptoms of GPA are constant fever, feeling exhausted all of the time, loss of appetite and weight loss even when the patient is not on a diet. Uncommon symptoms include a constantly running nose, nose pain, pain in the face, nose bleeds, coughing up bloody mucus or spittle, mild chest pain, shortness of breath, ear pain, ear inflammation, hearing loss, eye inflammation, eye bulging more than usual, vision problems, muscle pain, joint pain, skin rashes, skin sores, abdominal pain caused by inflamed kidneys, cloudy urine, pus in the urine, painful urination, nausea with or without vomiting and swelling of the hands or feet.
While the definitive cause of granulomatosis with polyangitis is not known, it is suspected that it may have something to do with a malfunction in the body’s immune responses. The National Organization for Rare Disorders (NORD) notes that the type of changes in bodily tissues affected by this disease, along with an overall increased immune response in the body, point to granulomatosis with polyangitis being an autoimmune disorder.
Some medical researchers believe that infections are part of the reason why the body develops this disorder. Antibodies are released to fight an infection but malfunction and end up attacking otherwise healthy tissues instead. In a type of granulomatosis with polyangitis, autoantibodies are produced that specifically attack healthy tissue. In another type of this same disease, the neutrophil type of white blood cells gives off an abnormally high amount of antibody immune proteins, so instead of destroying bacteria, they damage the body.
In either of these cases, this creates blood vessel inflammation that restricts ample blood flow, which then effects organ systems. Other theories for the cause of this rare disease are that genetics and the environment play a role in developing this condition.
A biopsy of the impacted area is often needed for a doctor to make a diagnosis. Blood tests, urinalysis and X-rays, MRIs or CT scans may be needed to confirm a diagnosis or rule out other causes of the patient’s symptoms.
Medications used to treat GPA include glucocorticoids like prednisone (a type of steroid), chemotherapy medications like cyclophosphamide and the biologic agent rituximab. More than one medication is usually prescribed. These can cause severe side effects but these side effects can often be treated. After GPA goes away there is a 50% to 70% percent chance it will come back but will go away again with treatment.
Harvard Medical School notes that there is no way to prevent granulomatosis with polyangitis at this time. It is known, however, that prompt treatment with strong anti-inflammatory medications can prevent death from this disorder. Continued use of anti-inflammatory medications can prevent a relapse of the disorder in most people and usher them into a state of remission.
The medical inquiry is ongoing into both the causes and prevention of this disease. Research into prevention of disease relapse has pointed to the fact that reducing the number of infections that sufferers of the disease get also decreases their rate of disease relapse.