Granulomatosis with Polyangiitis (GPA)
General Overview
General Overview
- Antineutrophil cytoplasmic autoantibody (ANCA)- associated vasculitis
- Previously known as Wegener's granulomatosis
- Other ANCA-associated vasculitides include
- Microscopic polyangiitis
- Eosinophilic granulomatosis with polyangitis (Churg-strauss)
- Symptoms
- Consitutional (fever, malaise, anorexia, weight loss)
- Eye (eye pain, visual changes, proptosis)
- ENT manifestations (sinusitis, otitis media, otorrhea/rhinorrhea, oral/nasal ulcers)
- Respiratory (hoarseness, cough, dyspnea, stridor, wheezing, hemoptysis, plerutic pain)
- Renal (glomerulonephritis - hematuria)
- Skin (purpura, necrosis, ulceration, urticaria, livedo reticularis, nodules)
- Diagnosis
- Positive ANCA with biopsy (for positive histology) to confirm
- Other investigations
- ANA, Anti-GBM, C3, C4, cryoglobulins
- Hepatitis, HIV, LFT, tuberculosis, blood cultures
- Serum creatinine (eGFR)
- UA, urine protein
- Consider CXR/CT chest
- Treatment
- Glucocorticoid WITH
- Methotrexate or rituximab or cyclophosphamide
- Glucocorticoid WITH