Granulomatosis with Polyangiitis Treatment Houston, Texas
- Granulomatosis with polyangiitis (Wegener’s) often affects the sinuses, lungs and kidneys, and can lead to kidney failure.
- To prevent complications, prompt diagnosis and treatment are essential.
- Recurrences of this form of vasculitis are common
Most commonly, GPA affects the sinuses, lungs and kidneys.
GPA belongs to a group of diseases characterized by blood vessel inflammation (vasculitis). In GPA, organ damage occurs as a result of inflammation in vasculitis involving the small- and medium-sized blood vessels, and from a type of tissue injury called granulomatous inflammation. [A granuloma is a type of inflammation that can, usually be seen on biopsies of affected organs.]
Most commonly, GPA affects the sinuses, lungs and kidneys, but also can affect the eyes, ears, skin, nerves, joints and other organs. Because of the variety of potential organ involvement, a wide range of symptoms can develop over days to months. For 90% of people, the first symptoms appear in the respiratory tract (e.g., nose, sinuses and lungs) and include nasal congestion, frequent nosebleeds, shortness of breath, and cough that may produce bloody phlegm. Other early symptoms can include joint pain, decreased hearing, skin rashes, eye redness and/or vision changes, fatigue, fever, appetite and weight loss, night sweats, and numbness or loss of movement in the fingers, toes or limbs.
Several well-studied therapies are effective in treating GPA. Although permanent organ damage can still occur, the medications used to treat GPA can improve or even resolve organ injury in many instances.
The diagnosis of GPA is based on clinical features affecting typical organ sites together with the identification of blood vessel and tissue inflammation in those organs. Because of this, most physicians will recommend a biopsy of the tissues involved to look under the microscope for evidence of characteristic changes.
Many people with GPA test positive for proteins in their blood called antineutrophil cytoplasmic antibodies, which is commonly called ANCA. A blood test positive for ANCA supports the need for further examination for GPA; it usually does not replace the need for a tissue biopsy. In rare instances where people have certain clinical features, a positive test may provide enough evidence to make a diagnosis of GPA. However, ANCA cannot be found in up to 20% of people with well-diagnosed GPA, so a negative ANCA test does not eliminate the possibility of GPA in patients with other characteristic clinical features.
Active GPA involving a critical organ site requires treatment to prevent ongoing inflammation that can result in organ failure. In the absence of treatment, GPA can worsen rapidly, leading to potentially life-threatening kidney or lung failure. Treatment choices are based on the organs affected, disease severity and individual medical factors.
Those with active severe disease often receive a glucocorticoid (steroid) medication such as prednisone combined with cyclophosphamide (Cytoxan), a chemotherapy type of medication. Prednisone is begun at a high dose and gradually reduced. Cyclophosphamide is given for 3-6 months. Those who improve are then switched to another medication such as methotrexate (Rheumatrex, Trexall) or azathioprine (Imuran, Azasan) for 2 or more years. Another recent option for the treatment of severe granulomatosis with polyangiitis is rituximab(Rituxan) combined with glucocorticoids. Rituximab is a biologic agent given by vein that targets a type of immune cell called the B lymphocyte. The patient and his or her doctor will work together to decide whether the patient should receive cyclophosphamide or rituximab for severe GPA. Those with active but not severe disease may be treated with prednisone and methotrexate. Each medication has significant side effects that must be considered to minimize or prevent the risk of serious side effects.
All of the medications used to treat GPA have significant side effects to consider. Minimizing or preventing the risk of serious side effects is an important part of caring for someone with GPA.
Even with effective treatment, recurrences (relapses) are common. Relapses may resemble or differ from the initial onset, so new symptoms should be reported to the medical practitioner as soon as possible. Regular doctor visits together with monitoring of laboratory tests and imaging studies can help to detect relapses early.
- GPA is serious but treatable.
- Significant side effects of medications used to treat GPA can be minimized with preventive strategies.
- While disease relapses frequently occur, early detection and treatment can lessen the potential for
serious organ injury.
Depending on the organ sites involved, people with GPA frequently require care from numerous physician specialists, each of whom contributes in a valuable way.