PANCE - Medium & Variable Vessel Vasculitis

Here are key facts for PANCE from the Medium & Variable Size Vessel Vasculitis tutorial, as well as points of interest at the end of this document that are not directly addressed in this tutorial but should help you prepare for the boards. See the tutorial notes for further details and relevant links.

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1. Vasculitis causes blood vessel inflammation, leading to ischemia, necrosis, and organ dysfunction across multiple systems. 2. First-line treatment for vasculitides usually includes high-dose corticosteroids. 3. Systemic symptoms include fever, weight loss, arthralgias, myalgias, and fatigue. 4. PAN is a necrotizing vasculitis of medium-sized muscular arteries. 5. It causes multisystem involvement:

• Nervous system: Mononeuritis multiplex (asymmetric neuropathy)
• Renal: Hypertension, renal ischemia (NO glomerulonephritis)
• GI: Mesenteric ischemia, abdominal pain
• Skin: Livedo reticularis, ulcers, nodules, gangrene

6. PAN is associated with hepatitis B infection (screen all patients). 7. Diagnosis:

• Biopsy shows necrotizing inflammation of medium arteries.
• Angiography shows microaneurysms and irregular vessel narrowing.

8. Treatment: High-dose corticosteroids; add antivirals and plasma exchange if HBV-positive. 9. Kawasaki disease is a medium-vessel vasculitis affecting mostly children <5 years. 10. Diagnostic criteria:

• Fever ≥5 days plus ≥4 of 5 findings:
• Bilateral non-purulent conjunctivitis
• Oral mucosal changes (strawberry tongue, fissured lips)
• Polymorphous rash
• Extremity changes (swelling, desquamation)
• Cervical lymphadenopathy

11. Major complication: Coronary artery aneurysms leading to myocardial infarction. 12. Treatment:

• IVIG plus high-dose aspirin.
• Repeat echocardiograms at diagnosis and at 2–6 weeks to monitor coronary arteries.

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1. Behçet disease is a variable-vessel vasculitis affecting arteries and veins of all sizes. 2. Clinical features:

• Oral aphthous ulcers
• Genital ulcers
• Ocular inflammation (anterior or posterior uveitis)
• Skin lesions (erythema nodosum, papulopustular rash)

3. Associated with HLA-B51 genetic marker. 4. Diagnosis is clinical; positive pathergy test (skin pustule formation after minor trauma) supports diagnosis. 5. Treatment:

• Corticosteroids during flares.
• Colchicine, azathioprine, or cyclosporine for maintenance and ocular disease.

6. Buerger disease affects small and medium arteries and veins, especially in young male smokers. 7. Key features:

• Distal ischemia causing claudication, coldness, numbness
• Ulceration and progression to gangrene
• Superficial thrombophlebitis

8. Diagnosis:

• Clinical suspicion in smokers.
• Arteriography shows corkscrew collaterals.

9. Management:

• Absolute smoking cessation is critical.
• No effective treatment if smoking continues.

10. PAN: Transmural necrotizing inflammation of medium arteries, no pulmonary involvement, ANCA-negative. 11. Kawasaki: Proliferative inflammation of coronary arteries. 12. Behçet: Neutrophilic vasculitis of arteries and veins. 13. Buerger: Occlusive thrombus with preserved vessel wall architecture.

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1. PAN spares the lungs, unlike GPA (Granulomatosis with Polyangiitis). 2. Cyclophosphamide is reserved for severe or refractory PAN not associated with hepatitis B. 3. Coronary aneurysm formation occurs within the first few weeks; early IVIG prevents it. 4. Patients should avoid live vaccines (e.g., MMR, varicella) for 11 months post-IVIG therapy. 5. DVTs and arterial thromboses can occur; important to monitor for signs of thrombotic complications. 6. Amputation may be necessary if tissue ischemia progresses despite smoking cessation. 7. Patients should be educated on good foot care and regular vascular checks.