Vasculitis syndromes: Difference between revisions
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==Background== | ==Background== | ||
[[File:Histopathology of giant cell arteritis.png|thumb|[[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 | *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 | *Presentation varies greatly depending on etiology and specific vessels affected | ||
*Classification by size of vessels predominantly involved | *Classification by size of vessels predominantly involved | ||
{{Primary Vasculitis DDX}} | |||
==Clinical Features== | ==Clinical Features== | ||
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*Bruits | *Bruits | ||
*[[Aortic regurgitation]] | *[[Aortic regurgitation]] | ||
*Sudden blindness (giant cell arteritis) | *Sudden [[vision loss|blindness]] (giant cell arteritis) | ||
===Medium Vessel Vasculitis=== | ===Medium Vessel Vasculitis=== | ||
*Cutaneous nodules, livedo reticularis | *Cutaneous nodules, livedo reticularis | ||
*Ulcers | *Ulcers | ||
*Digital gangrene | *Digital [[gangrene]] | ||
*Mononeuritis multiplex (asynchronous/asymmetric peripheral neuropathy) | *Mononeuritis multiplex (asynchronous/asymmetric peripheral neuropathy) | ||
*Microaneurysms | *Microaneurysms | ||
===Small Vessel Vasculitis=== | ===Small Vessel Vasculitis=== | ||
*[[Purpura]], urticaria, cutaneous necrotizing granulomas, splinter hemorrhages | *[[Purpura]], [[urticaria]], cutaneous necrotizing granulomas, splinter hemorrhages | ||
*[[Uveitis]] (ciliary spasm, visual changes, limbic redness), [[scleritis]]/[[episcleritis]] (ocular pain, photophobia) | *[[Uveitis]] (ciliary spasm, visual changes, limbic redness), [[scleritis]]/[[episcleritis]] (ocular pain, photophobia) | ||
*[[Glomerulonephritis]] ([[hematuria]], [[proteinuria]] | *[[Glomerulonephritis]] ([[hematuria]], [[proteinuria]] | ||
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==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{Purpura DDX}} | |||
==Evaluation== | ==Evaluation== | ||
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*Treat underlying illness, if known | *Treat underlying illness, if known | ||
*Manage critical complications: see treatment section of [[diffuse alveolar hemorrhage]], [[acute limb ischemia]], [[Kawasaki]] | *Manage critical complications: see treatment section of [[diffuse alveolar hemorrhage]], [[acute limb ischemia]], [[Kawasaki]] | ||
*For primary vasculitides, this will likely involve an | *For primary vasculitides, this will likely involve an immunosuppressive agent, such as: | ||
**[[Prednisone]]/[[Methylprednisolone]] | **[[Prednisone]]/[[Methylprednisolone]] | ||
**[[Cyclophosphamide]], [[methotrexate]], [[azathioprine]] | **[[Cyclophosphamide]], [[methotrexate]], [[azathioprine]] | ||
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==See Also== | ==See Also== | ||
*[[Immunocompromised antibiotics]] | |||
*[[Biologic immunomodulators]] | |||
==External Links== | ==External Links== | ||
Latest revision as of 18:19, 2 August 2023
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