From the Guidelines
GPA vasculitis is now referred to as Granulomatosis with Polyangiitis (GPA). This condition is characterized by necrotizing granulomatous inflammation and vasculitis, commonly associated with cytoplasmic ANCA and antibodies to proteinase 3 (PR3) 1. The clinical manifestations of GPA include destructive sinonasal lesions, pulmonary nodules, and pauci-immune glomerulonephritis.
Key Features of GPA
- Histologically characterized by necrotizing granulomatous inflammation in addition to vasculitis
- Common clinical manifestations include destructive sinonasal lesions, pulmonary nodules, and pauci-immune glomerulonephritis
- Most commonly associated with cytoplasmic ANCA and antibodies to proteinase 3 (PR3) 1
Diagnosis and Management
The diagnosis of GPA is based on a combination of clinical, laboratory, and histopathological findings. The management of GPA involves the use of immunosuppressive agents, including biologic and nonbiologic agents, to induce and maintain remission.
Current Understanding
The current understanding of GPA is based on recent clinical trials and observational studies that have investigated the efficacy and toxicity of different treatment regimens 1. These studies have provided evidence-based recommendations for the treatment and management of GPA, which is essential for improving patient outcomes and reducing morbidity and mortality.
From the FDA Drug Label
1.4 Granulomatosis with Polyangiitis (GPA) (Wegener's Granulomatosis) and Microscopic Polyangiitis (MPA) RITUXAN, in combination with glucocorticoids, is indicated for the treatment of adult and pediatric patients 2 years of age and older with Granulomatosis with Polyangiitis (GPA) (Wegener's Granulomatosis) and Microscopic Polyangiitis (MPA).
GPA vasculitis is now called Granulomatosis with Polyangiitis (GPA), previously known as Wegener's Granulomatosis 2.
From the Research
GPA Vasculitis
GPA vasculitis is now called Granulomatosis with Polyangiitis (GPA), formerly termed Wegener's Granulomatosis 3, 4, 5, 6, 7.
Key Characteristics
- GPA is a systemic necrotizing vasculitis that affects small- and medium-sized blood vessels and is often associated with cytoplasmic ANCA 4
- The main clinical characteristics involve the upper and/or lower respiratory tract and kidneys 4
- GPA can occur in all racial groups but predominantly affects Caucasians, and both sexes are affected equally 7
Clinical Manifestations
- Classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease, with the nasal cavity and the paranasal sinuses being the most common sites of involvement in the head and neck area 7
- Otological disease is found in approximately 35% of cases 7
- Lung nodules are frequently seen, sometimes excavated, and renal involvement is characterized by rapidly progressive necrotizing glomerulonephritis with extracapillary crescents 4
Diagnosis and Treatment
- Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis 7
- The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide, with rituximab being an effective remission-inducing agent in GPA 3, 6