Wegener Granulomatosis
| Overview |
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- Wegener Granulomatosis is a vasculitis of small to medium-sized vasculature, including arterioles, capillaries, and venules. The disease principally affects vasculature of the upper and lower respiratory tract along with the glomerular capillaries.
| Epidemiology |
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- The disease can manifest at any age although there is a predilection for middle-aged individuals. There does not appear to be a gender bias.
| Pathogenesis |
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- The pathogenesis of Wegener Granulomatosis is not well-understood. However, the disease is associated with ANCA auto-antibodies, specifically of the c-ANCA subtype.
| Morphology |
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- Respiratory Lesions
- Wegener Granulomatosis is characterized by a necrotizing vasculitis of both the upper and lower respiratory tract. Most often inflammation is associated with formation of intravascular or extravascular granulomas that contain giant cells. Coalescence of granulomas in the lung can cause cavitations that are visible by chest radiography.
- Renal Lesions
- Renal lesions initially manifest as focal, segmental inflammation of the glomerulus but often progress to Rapidly Progressive Glomerulonephritis. Because immune complexes are not observed, glomerular lesions are considered a Pauci-immune Glomerulonephritis.
| Clinical Consequences |
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- Most patients display respiratory and renal symptomology and without treatment death usually occurs within a year of diagnosis
- Upper Respiratory Tract: Chronic sinusitis, epistaxis, ulcerations of nasopharyngeal mucosa
- Lower Respiratory Tract: cough with hemoptysis along with chest pain
- Kidney: Hematuria and other signs of Nephritic Syndrome