From the Guidelines
Granulomatosis with polyangiitis (GPA) symptoms typically include persistent nasal congestion, nosebleeds, sinus pain, ear infections, and coughing up blood, as well as systemic symptoms such as fatigue, fever, night sweats, and weight loss, which can significantly impact quality of life 1.
Common Symptoms of GPA
- Nasal symptoms: crusting, discharge, nasal stuffiness, bleeding, reduced sense of smell, and facial pain
- Upper respiratory tract involvement: ear infections, hearing loss, vertigo, tinnitus, and facial nerve palsy
- Kidney involvement: blood or protein in urine, sometimes progressing to kidney failure
- Skin symptoms: rashes, purpura, and ulcers
- Eye inflammation: redness, pain, vision changes, or even blindness
- Joint and muscle pain, and neurological symptoms: numbness, weakness, or seizures
Diagnosis and Treatment
GPA is diagnosed through blood tests for ANCA antibodies, imaging studies, and tissue biopsies. Early diagnosis and treatment with immunosuppressive medications like cyclophosphamide, rituximab, corticosteroids, and maintenance drugs such as azathioprine or methotrexate are crucial to prevent permanent organ damage and improve prognosis 1.
Disease Activity and Quality of Life
Disease activity can be measured using the Birmingham Vasculitis Activity Score (BVAS), and quality of life is significantly affected in GPA, as shown by the general Short Form-36 and the Sinonasal Outcome Test-22 1.
Treatment Guidelines
The 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of ANCA-associated vasculitis recommends remission induction therapy with methotrexate, azathioprine, mycophenolate mofetil, cyclophosphamide, or rituximab, and remission maintenance therapy with methotrexate, azathioprine, mycophenolate mofetil, or rituximab 1.
From the Research
GPA Vasculitis Symptoms
The symptoms of GPA vasculitis are not explicitly described in the provided studies. However, the studies discuss the treatment and management of GPA vasculitis, which can be used to infer the severity and impact of the disease.
Treatment and Management
- The current treatment of GPA is stratified based on severity, with methotrexate and glucocorticoids used to induce and maintain remission in non-severe cases 2.
- For patients with severe disease, options include glucocorticoids combined with either cyclophosphamide or rituximab 2.
- Rituximab has been shown to be as effective as cyclophosphamide in inducing remission of severe active GPA 2, 3.
- Combination therapy with rituximab and cyclophosphamide has been used to induce remission in ANCA vasculitis, with a median time to complete remission of 4 months 4.
- Plasma exchange has been used in combination with rituximab and cyclophosphamide to treat severe ANCA-associated vasculitis, with a high rate of disease remission and recovery of independent kidney function 5.
Comparison of Treatment Options
- A study comparing rituximab and cyclophosphamide as induction therapy for GPA found that rituximab was more frequently associated with remission than cyclophosphamide 6.
- Another study found that rituximab was as effective as cyclophosphamide in treating patients with major renal disease or alveolar hemorrhage 3.
- The choice of treatment should be decided between the patient and physician, taking into account the severity of the disease and the potential side effects of each treatment option 2.