Granulomatosis with polyangiitis, is a disorder in which a dysregulated immune system causes inflammation of small blood vessels.
What is granulomatosis with polyangiitis?
Granulomatosis with polyangiitis, formerly known as Wegener’s granulomatosis, is a disorder in which a dysregulated immune system causes widespread inflammation of small blood vessels throughout the body. This results in slower or impaired blood flow to your nose, sinuses, throat, lungs, and kidneys.
Symptoms can be widespread and affect various parts of the body such as the eyes and ears or respiratory system, yet usually begin more generally with fever, fatigue, a loss of appetite, and weight loss. With treatment, a full recovery is possible; however, this condition can be fatal.
Treatments include immunosuppressive medications in order to control the disease in the short- and long-term.
You should visit your primary care physician soon to provide an early diagnosis. Treatment of inflammatory diseases commonly involve prescription medications such as a steroid to decrease inflammation, and a cell-damaging medicine to kill abnormal cells.
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Granulomatosis with polyangiitis symptoms
Because granulomatosis with polyangiitis is a systemic disease, it can cause a variety of symptoms. Furthermore, GPA can cause recurrent episodes with different sets of symptoms each time.
Most people with GPA will develop a period of main, general symptoms that may last for weeks or months before more specific symptoms develop. This may include:
Approximately 90 percent of people with GPA will develop some nasal symptoms, including:
- Runny nose
- Sinus congestion
- Nasal ulcers
- Crusting of the nose
- Saddle nose deformity: People with repeated inflammation of the nose can develop an abnormality called a "saddle nose deformity," in which the top of the nose sags inward.
Many people can also develop ear symptoms, including:
Respiratory and chest symptoms
Lung and chest-related symptoms are likely with granulomatosis with polyangiitis. These can include:
- Shortness of breath
- Noisy breathing
- Coughing up blood (hemoptysis): This symptom is less common but still occurs.
- Chest pain: This is usually sharp and may be worse when breathing in.
Kidney problems usually develop later in the course of the disease after the onset of other symptoms, and will appear as:
- Bloody urine (hematuria)
- Frothy urine
About one-half of people with this condition will develop skin symptoms in the form of various rashes, including:
- Purple-colored ulcers: A common type of skin rash is a purple discoloration with overlying ulcers, usually seen on the legs.
- Itchy wheals (urticaria)
- Red streaks
- Hard bumps in the skin
Some people with this condition can develop neurological symptoms, including:
- Numbness or weakness: This can occur in part of the face or body.
Some people with this condition can develop eye symptoms, such as:
Granulomatosis with polyangiitis causes
The specific cause of granulomatosis with polyangiitis is unclear but is thought to involve an initial inflammatory event that causes a dysregulated immune response. GPA commonly affects individuals who are elderly or white. Triggers for developing granulomatosis with polyangiitis include infection, exposure to a toxin, a reaction to certain medications, after developing a blood clot, or certain genetic variations.
Infections can cause a systemic inflammatory response throughout the body and may trigger the development of granulomatosis with polyangiitis. Specifically, Staphylococcus aureus, a common skin bacteria, is believed to potentially cause GPA and the associated widespread inflammation.
Exposure to toxins
These can also include inhaled irritants, such as:
- Silica: This is found in mining, sandblasting, and stonework.
Taking certain medications have been reported to trigger the development of granulomatosis with polyangiitis. A few possible medications and the conditions they are used for include:
- Hydralazine: For severely high blood pressure
- The antibiotic minocycline: For acne
- Propylthiouracil: For high thyroid levels
- Allopurinol: For Gout
- Rifampin: For tuberculosis
Developing a blood clot
Developing a blood clot can cause an inflammatory response in the body. They commonly develop in the leg and may be brought on by:
- Being immobile for a period of time: For example, taking a long plane ride
- Taking oral contraceptive pills (birth control pills)
Certain genetic variations have been associated with an increased risk of developing this condition. These include genetic variants of a group of molecules found on certain immune cells, which may be involved in the development of granulomatosis with polyangiitis.
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Granulomatosis with polyangiitis treatment
Granulomatosis with polyangiitis is a serious condition that is usually treated aggressively with medications to suppress the immune system until the active disease resolves. These medications are usually given in two phases: an initial "induction" phase and a subsequent "maintenance" phase.
"Induction" phase immunosuppressive medications
Most people with active GPA will be recommended to start with a course of "induction" immunosuppressive medications.
- Potency: These are stronger medications that will help quickly control the active inflammation.
- Duration: These medications are usually continued for three to six months.
- Medication types: Usually, this will consist of a steroid medication such as methylprednisolone and/or prednisone in combination with another medication such as methotrexate, rituximab, or cyclophosphamide.
"Maintenance" phase immunosuppressive medications
After a period of induction phase medications, most people with granulomatosis with polyangiitis will be switched to a course of maintenance phase immunosuppressive medications. The goal of maintenance therapy is to prevent the disease from causing symptoms again.
- Potency: These medications are generally less potent than the medications used during the induction phase, and thus may cause less long-term side effects.
- Duration: Maintenance therapy is usually continued for one to two years, and may be continued indefinitely in people who have had many episodes of disease.
- Medication types: Possible medication used for maintenance include methotrexate, rituximab, azathioprine (Imuran), or mycophenolate (Cellcept).
Additional treatment options
Some people with severe symptoms of GPA may benefit from a treatment called plasma exchange therapy or surgery to repair associated nasal deformities.
- Plasma exchange therapy: In this treatment, your blood is removed and replaced with blood products from a healthy donor. This can help quickly remove pro-inflammatory substances in the blood and control the active disease.
- Surgery to repair nasal deformities: People who develop nasal deformities such as the "saddle-nose deformity" may benefit from a surgery to repair the nose, which can help open up the nasal passages and result in a more normal-appearing nose.
When to seek further consultation
If you develop any symptoms of granulomatosis with polyangiitis such as fever, nosebleeds, nasal ulcers, ear pain, shortness of breath, rash, or blood in the urine, you should go to your physician. Your physician can order laboratory and imaging studies to determine if you have granulomatosis with polyangiitis, and start you on the appropriate treatment.
Questions your doctor may ask to diagnose GPA
- Have you been feeling more tired than usual, lethargic or fatigued despite sleeping a normal amount?
- Any fever today or during the last week?
- Have you experienced any nausea?
- Have you lost your appetite recently?
- Do you have trouble sleeping?
Self-diagnose with our free Buoy Assistant if you answer yes on any of these questions.
- Granulomatosis with polyangiitis. Mayo Clinic. Published August 8, 2017. Mayo Clinic Link
- Granulomatosis with polyangiitis (Wegener's). American College of Rheumatology. Updated May 2017. American College of Rheumatology Link
- Granulomatosis with polyangiitis. Genetic and Rare Diseases Information Center. GARD Link
- Granulomatosis with polyangiitis. National Organization of Rare Disorders. Published 2017. NORD Link
- Kubaisi B, Abu Samra K, Foster CS. Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations. Intractable Rare Dis Res. 2016;5(2):61-9. NCBI Link