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Vasculitis for the Nurse Practitioner Licensing Exam

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Overview of Vasculitis
    • Definition: Vasculitis is the inflammation of blood vessels, which can result in damage to the vessel walls, narrowing, or occlusion, leading to reduced blood flow and potential organ damage.
    • Classification:
  • Large vessel vasculitis: Affects large arteries (e.g., giant cell arteritis, Takayasu arteritis).
  • Medium vessel vasculitis: Affects medium-sized arteries (e.g., polyarteritis nodosa, Kawasaki disease).
  • Small vessel vasculitis: Affects small arteries, capillaries, and venules (e.g., granulomatosis with polyangiitis, microscopic polyangiitis, Henoch-Schönlein purpura).
    • Etiology:
    • Idiopathic in most cases but can be secondary to infections, autoimmune disorders, medications, or malignancies.
Clinical Features
    • General Symptoms:
  • Systemic symptoms such as fever, fatigue, weight loss, and myalgia.
  • Organ-specific symptoms depend on the type of vasculitis and the affected organs.
    • Large Vessel Vasculitis:
    • Giant cell arteritis: Temporal headache, jaw claudication, scalp tenderness, visual disturbances.
    • Takayasu arteritis: Limb claudication, absent pulses, differences in blood pressure between limbs.
    • Medium Vessel Vasculitis:
    • Polyarteritis nodosa (PAN): Abdominal pain, hypertension, kidney damage, peripheral neuropathy.
    • Kawasaki disease: Prolonged fever, conjunctivitis, mucous membrane changes, coronary artery aneurysms.
    • Small Vessel Vasculitis:
    • Granulomatosis with polyangiitis (GPA): Upper respiratory tract symptoms, lung involvement, glomerulonephritis.
    • Microscopic polyangiitis (MPA): Pulmonary hemorrhage, renal involvement.
    • Henoch-Schönlein purpura (HSP): Palpable purpura, abdominal pain, arthritis, renal involvement.
Diagnosis
    • Laboratory Tests:
  • Increased ESR and CRP indicate systemic inflammation.
  • Autoantibodies: ANCA (positive in GPA, MPA), elevated IgA (in HSP).
  • Basic lab results may show anemia, leukocytosis, and elevated creatinine in renal involvement.
    • Imaging:
    • Angiography: Useful in diagnosing medium and large vessel vasculitis by detecting aneurysms or stenosis.
    • CT/MRI: Helps in identifying structural changes in the affected vessels.
    • Biopsy:
  • Considered the definitive method for diagnosing small vessel vasculitis.
Treatment
    • Corticosteroids:
  • High-dose corticosteroids are the first-line treatment for most vasculitis types.
  • Gradual tapering is necessary after symptom control.
    • Immunosuppressive Therapy:
  • Cyclophosphamide, methotrexate, azathioprine for severe or refractory cases.
  • Rituximab: Often used in ANCA-associated vasculitis (GPA, MPA).
    • Special Treatments:
    • IVIG: Used for Kawasaki disease to prevent coronary artery complications.
    • Tocilizumab: Approved for use in giant cell arteritis.
Essential Points
    • Vasculitis is classified based on the size of the vessels involved (large, medium, small).
    • Diagnosis is based on clinical findings, lab results (e.g., ANCA, ESR), imaging, and biopsy when needed.
    • Early treatment with corticosteroids and immunosuppressants can prevent severe organ damage.
    • Rapid identification and management are essential in diseases like giant cell arteritis (risk of blindness) and Kawasaki disease (risk of coronary artery aneurysms).