Medium & Variable-Sized Vasculitis for the Nurse Practitioner Licensing Exam
Medium and variable-sized vasculitis includes
Polyarteritis Nodosa (PAN),
Kawasaki Disease,
Thromboangiitis Obliterans (Buerger’s Disease), and
Behçet’s Disease. These diseases can affect medium-sized vessels, with Behçet’s Disease involving both arteries and veins.
Polyarteritis Nodosa (PAN)
- Definition:
- A systemic necrotizing vasculitis affecting medium-sized arteries, often involving the renal, GI, and nervous systems.
- Clinical Presentation:
- Systemic Symptoms: Fever, weight loss, and fatigue.
- Renal: Hypertension, renal ischemia.
- Gastrointestinal: Abdominal pain and potential for bowel infarction.
- Neurologic: Mononeuritis multiplex (asymmetric neuropathies).
- Diagnosis and Management:
- Biopsy shows transmural inflammation, and angiography may show microaneurysms.
- Treated with corticosteroids and immunosuppressive therapy.
Kawasaki Disease
- Definition:
- An acute vasculitis in children, primarily affecting the coronary arteries.
- Clinical Presentation:
- Fever >5 days, conjunctivitis, strawberry tongue, rash, extremity erythema, and cervical lymphadenopathy.
- Complications:
- Coronary artery aneurysms can lead to myocardial infarction if untreated.
- Management:
- Treated with IVIG and high-dose aspirin to reduce aneurysm risk.
Thromboangiitis Obliterans (Buerger’s Disease)
- Definition:
- A non-atherosclerotic vasculitis affecting small- and medium-sized vessels, strongly linked to smoking.
- Clinical Presentation:
- Distal ischemia, pain, ulcers, and Raynaud’s phenomenon in extremities.
- Management:
- Requires smoking cessation to halt progression; symptomatic care as needed.
Behçet’s Disease
- Definition:
- A variable vessel vasculitis with recurrent oral and genital ulcers, uveitis, and skin lesions.
- Clinical Presentation:
- Painful ulcers, uveitis, skin lesions (erythema nodosum), and risk of venous thrombosis.
- Management:
- Managed with corticosteroids for flares and immunosuppressants for chronic symptoms.
Key Points
- Polyarteritis Nodosa (PAN): Causes systemic symptoms, renal hypertension, GI ischemia; treated with corticosteroids.
- Kawasaki Disease: Pediatric vasculitis with prolonged fever, mucocutaneous symptoms, and risk of coronary aneurysms; managed with IVIG and aspirin.
- Thromboangiitis Obliterans (Buerger’s Disease): Occurs in smokers, causing extremity ischemia; smoking cessation is critical.
- Behçet’s Disease: Presents with recurrent oral/genital ulcers and uveitis; treated with corticosteroids and immunosuppressants for severe cases.