Wegeners

Wegener's granulomatosis, now more commonly referred to as granulomatosis with polyangiitis (GPA), is a form of necrotizing vasculitis that mainly affects small- to medium-sized vessels. The disease is characterized by granulomatous inflammation and can involve multiple organs, most notably the upper and lower respiratory tracts and the kidneys. The term Wegener's granulomatosis is a historical eponym; due to historical associations of the eponym's namesake, many guidelines prefer the non-eponym GPA.

Clinical features typically reflect involvement of the respiratory system and kidneys. Patients may experience chronic sinusitis, nasal crusting, epistaxis, otitis media, cough, shortness of breath, chest pain, and hemoptysis. Kidney involvement can present as rapidly progressive glomerulonephritis, leading to rising creatinine and proteinuria. Constitutional symptoms such as fever, fatigue, weight loss, and night sweats are common. The course is variable, with periods of remission and relapse.

Diagnosis relies on a combination of clinical assessment, laboratory testing, imaging, and tissue biopsy. The presence of anti-neutrophil cytoplasmic antibodies, particularly cytoplasmic ANCA with PR3 specificity (c-ANCA/PR3-ANCA), supports the diagnosis but is not universal. Biopsy of involved tissue showing necrotizing granulomatous inflammation is confirmatory. Imaging, such as chest CT, may reveal nodules or infiltrates.

Treatment usually involves induction therapy with high-dose glucocorticoids plus either cyclophosphamide or rituximab, followed by maintenance therapy with agents such as azathioprine or methotrexate. Early and sustained treatment improves prognosis, but relapse and treatment-related complications, including infections, remain concerns.