Vasculitis

Aka: Vasculitis

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II. Definition

  1. Primary disorder of vascular inflammation

III. Epidemiology

  1. Cutaneous Small Vessel Vasculitis is most common

IV. Types

  1. Large-Vessel Vasculitis
    1. Giant Cell Arteritis (Carotid Artery)
    2. Takayasu's Disease (Aorta)
  2. Medium-Vessel Vasculitis
    1. Polyarteritis Nodosa
    2. Kawasaki's Disease (coronary arteries)
  3. Small-Vessel Vasculitis (ANCA or Non-ANCA associated)
    1. Granulomatosis with Polyangiitis (respiratory tract, Glomerulonephritis)
      1. Previously known as Wegener's Granulomatosis
    2. Churg-Strauss Syndrome (respiratory tract)
    3. Microscopic Polyangiitis (respiratory tract, Glomerulonephritis)
    4. Henoch-Schonlein Purpura (IgA deposits in skin, GI Tract, joints, Glomerulonephritis)
    5. Essential Cryoglobulinemia (skin, glomeruli)
    6. Cutaneous leuokocytoclastic Vasculitis (skin only)

V. Associated Conditions

  1. Headache
  2. Sinusitis
  3. Mononeuritis multiplex
  4. Cerebrovascular Accident
  5. Hematuria

VI. Signs: General

  1. Fever
  2. Hypertension
  3. Weight loss
  4. Rales on pulmonary exam
  5. Hepatomegaly or Splenomegaly
  6. Palpable Peripheral Nerves
  7. Skin findings (see specific findings based on type below)
    1. Superficial Thrombophlebitis
    2. Skin Ulcerations

VII. Signs: Specific findings based on type

  1. Large Vessel Vasculitis
    1. Arterial Bruit
    2. Pulsation loss
  2. Medium Vessel Vasculitis
    1. Nodules
    2. Necrotizing Livedo Reticularis
  3. Small Vessel Vasculitis
    1. Palpable Purpura
    2. Urticaria

VIII. Labs

  1. Cutaneous lesional Punch Biopsy is single most important study
    1. Biopsy the most characteristic, early active lesions
    2. Send for histopathology and direct immunofluorescence
  2. Inflammatory markers (acute phase reactants) and Infectious disease testing
    1. Complete Blood Count (CBC)
    2. Erythrocyte Sedimentation Rate (ESR)
    3. C-Reactive Protein (C-RP)
    4. Blood Culture
    5. Site-specific culture
  3. Renal tests
    1. Urinalysis
    2. Serum Creatinine
    3. Blood Urea Nitrogen (BUN)
    4. Creatinine phosphokinase (CPK)
  4. Hepatic tests
    1. Liver Function Tests (hepatic panel)
    2. Hepatitis B Surface Antigen
    3. Hepatitis C Antibody
    4. Cryoglobulin
  5. Rheumatologic tests
    1. Antinuclear Antibody
    2. Anticardiolipin Antibody
    3. Rheumatoid Factor
    4. Serum complement
    5. Anti-dsDNA
    6. Extractable nuclear Antigen
    7. Serum Protein Electrophoresis
    8. Antineutrophil cytoplasmic autoantibodies (ANCA)
      1. Reflex if positive to Proteinase 3 or myeloperoxidase

IX. Imaging

  1. Chest XRay or Chest CT
  2. Angiography (CTA or MRA)

X. Diagnostics: If Indicated

  1. Electrocardiogram
  2. Echocardiogram
  3. Electromyogram (EMG)

XI. Differential Diagnosis

  1. Antiphosphospholipid Antibody syndrome
  2. Atheroembolic Disease
  3. Cocaine Abuse
  4. Amphetamine Abuse
  5. Hypersensitivity Reaction
  6. Endocarditis
  7. Multiple Myeloma
  8. Sick Cell Anemia
  9. Secondary Vasculitis
    1. Rheumatoid Arthritis
    2. Systemic Lupus Erythematosus
    3. Scleroderma
    4. Hepatitis B Infection
    5. Hepatitis C Infection
    6. Lymphioma
    7. Solid organ tumor

XII. References

  1. Bohjanen (2009) UMN CME Internal Medicine Review, Minneapolis
  2. Jennette (1994) Arthritis Rheum 37:187 [PubMed]
  3. Sharma (2011) Am Fam Physician 83(5): 556-65 [PubMed]

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