Lecture Notes on Vasculitis

Overview

• Vasculitis is a group of disorders characterized by inflammation of blood vessels.
• Divided into large, medium, and small vessel vasculitis.
• Key focus on Giant Cell Arteritis (GCA) and Takayasu Arteritis for large vessels; Kawasaki Disease, Thromboangiitis Obliterans, and Polyarteritis Nodosa for medium vessels; and several types for small vessels.

Large Vessel Vasculitis

Giant Cell Arteritis (GCA)

• Symptoms:
  • Elevated ESR
  • Jaw claudication
  • Temporal headaches
  • Visual problems due to ophthalmic artery involvement
  • Associated with polymyalgia rheumatica
• Diagnosis and Treatment:
  • Classically in individuals over 50 with symptoms during activities like chewing
  • High-dose corticosteroids immediately before biopsy to prevent blindness.
• Mnemonic: GCA - Give Corticosteroids Immediately.

Takayasu Arteritis

• Features:
  • Transmural fiber thickening of aortic branches, notably affecting subclavian arteries.
  • Known as "pulseless disease" due to decreased blood pressure in upper extremities.
• Demographics:
  • Typically affects Japanese women under 40.
• Mnemonic: Samurai losing an arm reminds of perfusion issues in upper extremities.

Medium Vessel Vasculitis

Kawasaki Disease

• Characteristics:
  • Necrotizing vasculitis involving coronary arteries in children.
  • Risk of myocardial infarction (MI) in children.
  • Symptoms: Adenopathy, oropharyngeal signs, desquamating rash.
• Treatment:
  • Administer IV immunoglobulin and aspirin (unique due to risk of Reye’s syndrome).
• Mnemonic: COW that MOOD - M for MI, O for Adenopathy, O for Oropharyngeal signs, D for Desquamation.

Thromboangiitis Obliterans (Buerger's Disease)

• Key Points:
  • Distal digital thrombosis in men aged 30-50 who are chronic smokers.
  • Treatment involves cessation of smoking.

Polyarteritis Nodosa

• Details:
  • Transmural arteritis with fibrinoid necrosis.
  • String of pearls appearance on biopsy.
  • 30% of patients have Hepatitis B surface antigen.
• Mnemonic: Plan B, give pearls (string of pearls) and association with Hepatitis B.

Small Vessel Vasculitis

Henoch-Schönlein Purpura (IgA Vasculitis)

• Commonality:
  • Hematuria, palpable purpura, arthralgias.
  • GI pain following URI, associated with IgA deposition.

Cryoglobulinemic Vasculitis

• Characteristics:
  • Similar symptoms to IgA vasculitis but no GI pain.
  • Due to Hepatitis C infection; treat underlying condition.
• Mnemonic: HSP - Stomach Pain ; CGV - C gets Viral (Hepatitis C).

Churg-Strauss Syndrome

• Also Known As: Eosinophilic Granulomatosis with Polyangiitis
• Features:
  • Associated with asthma, allergic rhinitis, nasal polyps.
  • Positive for p-ANCA; peripheral neuropathy may occur.
• Mnemonic: Chug Strauss Syndrome - Pabst (P-ANCA, Asthma, Balls in Nose, Sensory deficits, T-IGE antibodies).

Wegener's Granulomatosis (Granulomatosis with Polyangiitis)

• Involves:
  • SI-ANCA positive.
  • Affects lungs, kidneys, nasopharynx.
• Mnemonic: C for crossing involvement - nasopharynx, lungs, kidneys.

Microscopic Polyangiitis

• Characteristics:
  • P-ANCA positive.
  • Involves lungs and kidneys but not nasopharynx.
• Mnemonic: P crosses lungs and kidney on a stick figure.

Conclusion:

• This overview covered key points, mnemonics, and treatments for high-yield vasculitis topics relevant to medical exams.