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Pathology- Vasculitis
Definition: Vasculitis encompasses a group of diseases where inflammation and damage to blood vessels are the primary pathology. This damage can affect vessels of varying sizes, leading to diverse clinical presentations.
Types of Vasculitis:
This guide outlines key features to differentiate between the vasculitides discussed. Remember that overlap exists, and accurate diagnosis often requires biopsy and serological testing (ANCA).
1. Giant Cell (Cranial or Temporal) Arteritis:
Definition: Vasculitis encompasses a group of diseases where inflammation and damage to blood vessels are the primary pathology. This damage can affect vessels of varying sizes, leading to diverse clinical presentations.
Types of Vasculitis:
This guide outlines key features to differentiate between the vasculitides discussed. Remember that overlap exists, and accurate diagnosis often requires biopsy and serological testing (ANCA).
1. Giant Cell (Cranial or Temporal) Arteritis:
- Vessel Size: Medium and large arteries.
- Location: Primarily head and neck arteries (temporal artery most commonly).
- Demographics: Adults >50 years old.
- Presentation: Weeks to months of fever, anorexia, weight loss, headache, scalp tenderness, jaw claudication (pain with chewing).
- Serious Complications: Ocular involvement (blindness), aortic aneurysm (25% of cases).
- Diagnosis: Temporal artery biopsy (positive in only 60% due to focal nature of inflammation); shows lymphohistiocytic infiltrate, media disruption, and often giant cell reaction.
- Vessel Size: Medium-sized arteries.
- Presentation: Systemic vasculitis leading to aneurysm formation and vessel narrowing. Relatively rare with strict diagnostic criteria.
- Organ Involvement: Gastrointestinal tract (abdominal pain), nervous system (peripheral nerve palsies), muscles (muscle aches).
- Diagnosis: Imaging showing vessel narrowing and aneurysms; biopsy confirming necrotizing vasculitis.
- Vessel Size: Small vessels.
- Presentation: Systemic vasculitis with prominent upper respiratory tract, lung, and kidney involvement. Characterized by c-ANCA positivity.
- Symptoms: Nasal symptoms, acute renal failure, pulmonary symptoms.
- Diagnosis:
- Renal biopsy: Focal segmental necrotizing glomerulonephritis with crescent formation (identical to microscopic polyangiitis).
- Lung biopsy: Large necrotizing granulomatous inflammation and necrotizing vasculitis.
- Treatment: Aggressive immunosuppression is crucial to prevent death.
- Vessel Size: Small vessels.
- Presentation: Systemic vasculitis with significant renal and lung involvement. Characterized by p-ANCA positivity.
- Demographics: Adults, median age 55.
- Symptoms: Acute renal failure, pulmonary symptoms.
- Diagnosis:
- Renal biopsy: Focal segmental necrotizing glomerulonephritis with crescent formation (identical to Wegener's granulomatosis).
- Lung biopsy: Marked alveolar hemorrhage and necrotizing capillaritis within alveolar septa.
- Treatment: Aggressive immunosuppression is essential for survival.
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