Vasculitis syndromes

Background

• Heterogenous group of disorders, clinico-pathologic process characterized by inflammation within walls of blood vessels, compromise of vessel lumen, and resulting ischemia
• Presentation varies greatly depending on etiology and specific vessels affected
• Classification by size of vessels predominantly involved
• Large-vessel:
  • Takayasu arteritis
  • Giant cell arteritis (temporal arteritis)
• Medium-vessel:
  • Kawasaki disease
  • Polyarteritis nodosa
  • Thromboangiitis obliterans (Buerger disease)
  • Primary angiitis of the central nervous system
• Small-vessel:
  • Henoch-Schönlein purpura
  • ANCA-associated vasculitides: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis
  • Cutaneous leukocytoclastic angiitis (“hypersensitivity vasculitis”)
  • Essential cryoglobulinemia, cryoglobulinemic vasculitis due to hepatitis C
  • Behcet's disease
• Secondary vasculitides and other/miscellaneous
  • Drug-induced vasculitis
  • Serum sickness
  • Vasculitis associated with other rheumatic diseases

Clinical Features

• Fever
• Arthralgias/arthritis
• Malaise, fatigue, weight loss

Large Vessel Vasculitis

• Limb claudication
• Asymmetric blood pressures
• Diminished or absent peripheral pulses
• Bruits
• Aortic regurgitation
• Sudden blindness (giant cell arteritis)

Medium Vessel Vasculitis

• Cutaneous nodules, livedo reticularis
• Ulcers
• Digital gangrene
• Mononeuritis multiplex (asynchronous/asymmetric peripheral neuropathy)
• Microaneurysms

Small Vessel Vasculitis

• Purpura, urticaria, cutaneous necrotizing granulomas, splinter hemorrhages
• Uveitis (ciliary spasm, visual changes, limbic redness), scleritis/episcleritis (ocular pain, photophobia)
• Glomerulonephritis (hematuria, proteinuria
• Hemoptysis, Diffuse alveolar hemorrhage

Differential Diagnosis

Petechiae/Purpura (by cause)

• Abnormal platelet count and/or coagulation
  • Septicemia
  • Idiopathic thrombocytopenic purpura (ITP)
  • Hemolytic uremic syndrome
  • Leukemia
  • Coagulopathies (e.g. hemophilia)
  • Henoch-Schonlein Purpura (HSP)
  • Acute hemorrhagic edema of infancy (AHEI)
• Hypersensitivity vasculitis
• Primary vasculitides
  • Wegener's
  • Microscopic polyangiitis
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
• Secondary vasculitides
  • Henoch-Schonlein purpura
  • Connective tissue disorder
    • Systemic lupus erythematosus
  • Scurvy
  • Infectious disease
    • Hepatitis B
    • Hepatitis C
• Trauma

Evaluation

• Workup dependent on presentation, likely to include at least CBC, ESR, BMP, UA

Management

• Treat underlying illness, if known
• Manage critical complications: see treatment section of diffuse alveolar hemorrhage, acute limb ischemia, Kawasaki
• For primary vasculitides, this will likely involve an immunosupressive agent, such as:
  • Prednisone/Methylprednisolone
  • Cyclophosphamide, methotrexate, azathioprine
  • Rituximab
• May consider plasmapheresis for severe or rapidly progressive