What Is the Treatment for Granulomatosis with Polyangiitis?
The goal of treatment for granulomatosis with polyangiitis is to bring about remission, maintain remission, and treat the disease if it becomes active again (relapse).
Treatment for granulomatosis with polyangiitis with medication can bring long-term remission for most people. Remission means the disease disappears or its progression is slowed, but the disease is not cured.
Without medical treatment, a person diagnosed with granulomatosis with polyangiitis has a high risk of dying of the disease within two years, usually from lung or kidney failure.
- People with severe granulomatosis with polyangiitis need urgent care because their condition is life-threatening.
- A less sever form is called limited granulomatosis with polyangiitis.
Both types are treated with similar medications. Surgery may be necessary.
Because granulomatosis with polyangiitis affects so many different parts of the body, a healthcare team of doctors who specialize in lung disease (pulmonologists), kidney disease (nephrologists), arthritis, and similar conditions (rheumatologists), and ear, nose, and throat diseases (otolaryngologists).
Remission is likely in most people when treated with medications. Most respond to the drug cyclophosphamide (Cytoxan), and a majority have complete remission.
In up to half of people who respond to a course of medications, the disease eventually becomes active again (relapse), and then therapy is begun again.
- To bring about remission, people with severe granulomatosis with polyangiitis may be given corticosteroids, for example, prednisone, for the first few months and cyclophosphamide, which is taken by pill form for up to a year. Sometimes cyclophosphamide may be given once per month through an IV injection (called pulse therapy). Blood tests are needed every two weeks to check for a low white blood cell count, which could weaken the immune system and cause infection easily.
- People with limited granulomatosis with polyangiitis may be treated with steroids and with drugs called immunosuppressive agents (such as methotrexate) to bring about remission.
- A person who has a relapse may be given cyclophosphamide and prednisone and possibly an antibiotic.
These are powerful medications and may cause toxic side effects. It is important to know what to expect during treatment and discuss all side effects with the doctor. With careful monitoring, side effects can be reduced.
Side effects of these drugs:
- Prednisone: Ideally, corticosteroids are used for only a short time in order to bring sudden flares in symptoms under control. Long-term use is associated with serious side effects, such as osteoporosis, glaucoma, cataracts, mental changes, abnormal blood glucose levels, or arrested bone growth in children before puberty. After prolonged use, the corticosteroid dose must be gradually decreased over weeks to months to avoid corticosteroid withdrawal syndrome.
- Cyclophosphamide: Blood cell counts are monitored regularly to watch for toxic effects. The urine is examined regularly for red blood cells, which may be the first sign of hemorrhagic cystitis (severe inflammation of the bladder that causes blood in the urine). It is important to drink lots of fluids while on this drug to prevent hemorrhagic cystitis.
- Methotrexate: To guard against problems, kidney and liver function are monitored on a regular basis, as are blood cell counts. Methotrexate may cause toxic effects on blood, kidneys, liver, lungs, and gastrointestinal and nervous systems.
- Inflamed tissue may show necrosis (localized tissue death) and need to be surgically removed. Such tissue would be located in the nose, throat, and lungs. In the nose, this may cause frequent nosebleeds and a perforated nasal septum.
- Problems with middle ear infections can be remedied by surgically inserting ear tubes through the eardrum to allow the ear to drain.
- For people with severe kidney failure, a kidney transplant may be a lifesaving option.