Info
🌱 來自: Huppert’s Notes
Medium Vessel Vasculitis🚧 施工中
Medium Vessel Vasculitis
Polyarteritis nodosa (PAN)
• Epidemiology: Incidence 3–4.5/100,000. Average age of onset 50 yr, M>F. Only 5% of cases are associated with HBV in the post-HBV vaccine era
• Clinical features:
- Neuro (75–80%): Mononeuritis multiplex or distal symmetric polyneuropathy
- Constitutional (65%): Fever, malaise, weight loss
- Cutaneous (50–60%): Livedo reticularis (lace-like rash), nodules, necrotic ulcers of the lower extremities
- Renal (40%): Can lead to hypertension, AKI/CKD if bilateral, hematuria, proteinuria
- GI (35–40%): Acute or chronic mesenteric ischemia
• Diagnosis:
- Diagnosis confirmed with biopsy of involved tissue (e.g., sural nerve) showing focal segmental panmural necrotizing inflammation
- If there are no clear biopsy targets but GI or renal involvement is suspected, mesenteric or renal angiography can show a combination of aneurysms and stenoses at vessel branch points
- Avoid renal biopsy given high risk of bleeding with aberrant vasculature
- Inflammatory markers are typically elevated
• Treatment:
- Prednisone and cyclophosphamide for severe non-HBV associated cases
- Antivirals for HBV-related disease
Primary angiitis of the central nervous system (PACNS)
• Epidemiology: Incidence is 2.4/100,000 annually. Median age of onset is 50 yr
• Clinical features: Medium vessel vasculitis with isolated CNS involvement. Presents with triad of headache, cognitive impairment or altered mental status, and new or progressive focal neurologic deficits due to strokes
• Diagnosis: MRA or conventional angiography may show beading of CNS artery branches; the gold standard for diagnosis is a brain or leptomeningeal biopsy showing lymphocytic vessel wall inflammation
• Treatment: High-dose glucocorticoids and cyclophosphamide. Permanent disability commonly occurs due to strokes.
Kawasaki disease
• Clinical features:
- Medium vessel vasculitis most common in children (80% of cases present prior to age 5 yr), typically Asian boys
- Manifestations include high fevers, conjunctivitis, mucositis (strawberry tongue), non-suppurative cervical adenitis, truncal rash, and plamar/plantar erythema
- Coronary aneurysms occur in 25% of cases
• Treatment:
- IVIG and aspirin. Excellent prognosis
- Coronary aneurysms may develop and cause CHF and/or need for CABG in adulthood