Vasculitis syndromes
Background
Giant cell arteritis on histopathology.
- 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
Vasculitis Syndrome Types
- Large vessel
- Takayasu arteritis
- Giant cell arteritis (temporal arteritis)
- Medium-vessel
- Kawasaki disease
- Polyarteritis nodosa
- Thromboangiitis obliterans (Buerger's disease)
- Primary angiitis of the central nervous system
- Small-vessel
- Henoch-Schönlein purpura
- ANCA-associated vasculitides
- Granulomatosis with polyangiitis (Wegner's)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
- Cutaneous leukocytoclastic angiitis (“hypersensitivity vasculitis”)
- Essential cryoglobulinemia, cryoglobulinemic vasculitis due to hepatitis C
- Behçet's disease]
- Secondary vasculitides and other/miscellaneous
- Drug-induced vasculitis
- Serum sickness
- Vasculitis associated with other rheumatic diseases (e.g. SLE)
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
- 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 immunosuppressive agent, such as:
- May consider plasmapheresis for severe or rapidly progressive