What is granulomatosis with polyangiitis (GPA)?
Granulomatosis with polyangiitis (GPA) is an uncommon type of inflammation of small arteries and veins (vasculitis). It classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages (sinuses), and the kidneys. "Incomplete" forms exist that only involve one of these areas. When both lungs and kidneys are affected, the condition is sometimes referred to as generalized granulomatosis with polyangiitis. When only the lungs are involved, the condition is sometimes referred to as limited granulomatosis with polyangiitis. The terminology "granulomatosis with polyangiitis" highlights the two central pathologic features of the disease, granuloma formation and inflammation of blood vessels (angiitis or vasculitis).
The name "Wegener's granulomatosis" is no longer used because the German physician Friedrich Wegener, the disease's former namesake, was a member of the Nazi party and allegedly linked to Nazi war crimes.
Granulomatosis with polyangiitis usually affects young or middle-aged adults. Although it is uncommon in children, it can affect people at any age. The cause of the condition is not known.
What are symptoms of granulomatosis with polyangiitis?
Symptoms of granulomatosis with polyangiitis include:
- bloody sputum,
- joint pains,
- shortness of breath,
- sinus inflammation (sinusitis), and
- weight loss.
Nasal ulcerations and even bloody nasal discharge can occur. Other areas of the body that can also become inflamed in patients with GPA include the eyes, the nerves (neuropathy), the middle ear (otitis media), and the skin resulting in skin nodules or ulcers.
Diagnosis of granulomatosis with polyangiitis
Abnormal lab findings in patients with granulomatosis with polyangiitis include urine tests that detect protein and red blood cells in the urine (not visible to the naked eye) and X-rays of the chest and sinuses, which detect abnormalities resulting from lung and sinus inflammation. Blood tests that detect the abnormal inflammation include the sedimentation rate (sed rate) and C-reactive protein. A more specific blood test used to diagnose and monitor granulomatosis with polyangiitis is the antineutrophil cytoplasmic antibody (ANCA test), which is commonly elevated when the disease is active.
The diagnosis of granulomatosis with polyangiitis is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis in a biopsy of tissue involved with the inflammatory process. For examples, an open lung biopsy or a kidney biopsy are commonly used in making a diagnosis of the condition.
What is the treatment for granulomatosis with polyangiitis?
Granulomatosis with polyangiitis is a serious disease and without treatment can be fatal within months. Treatment is directed toward stopping the inflammation process by suppressing the immune system.
- Medications used to treat granulomatosis with polyangiitis include high-dose cortisone (prednisone) and the immunosuppressive drug cyclophosphamide (Cytoxan). Recent reports also suggest that trimethoprim/sulfamethoxazole (Bactrim) also can be helpful to prevent relapse of disease activity in patients with the condition.
- Cytoxan that is taken by mouth with prednisone until the disease is in remission and then switched to methotrexate (Rheumatrex, Trexall) for two years and tapered off has been reported to be effective and less toxic than the traditional long-term Cytoxan treatment.
- Methotrexate (Rheumatrex, Trexall) has been introduced as a drug for Cytoxan treatment failures. Moreover, it now appears that Cytoxan will not be necessary in order to maintain long-term remission, and that doctors can convert to the less toxic methotrexate for maintenance.
- Reports also demonstrate that methotrexate can eventually be tapered off entirely after two years. Azathioprine (Imuran) has also been used as a maintenance medication after Cytoxan.
- Intravenous immunoglobulin therapy (IVIG) has been shown to be helpful in treating relapses of granulomatosis with polyangiitis.
A newer and more effective treatment for granulomatosis with polyangiitis is rituximab (Rituxan), given intravenously weekly for four weeks in combination with cortisone medication. These new regimens are welcome news for patients with as medical researchers are searching for better treatments.
What is the prognosis for granulomatosis with polyangiitis?
The prognosis for granulomatosis with polyangiitis depends on which organs are involved, to what degree they are involved, the duration of the disease, and the response to treatment. Today, with an optimal response to treatment, people with the condition scan lead normal lives.
Kelley and Firestein's Textbook of Rheumatology, 10th Edition. 2016.
Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.
Tracy, CL, et al. "Granulomatosis With Polyangiitis (Wegener Granulomatosis)." Medscape. Dec. 2, 2017. <https://emedicine.medscape.com/article/332622-overview>.
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