Names for vasculitides adopted by the 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides
About
- Multiple conditions causing inflammation of blood vessels
- See individual diseases for more information
Clinical
- Most present with fatigue, malaise, fever, weight loss
- Arthralgia, arthritis, myalgia
- Glomerulonephritis
- Lungs: alveolitis, alveolar haemorrhage, sinusitis
- Neuropathy: mononeuritis multiplex, sensory neuropathy
- Jaw claudication and visual loss
- Abdominal pain, intestinal perforation, diarrhoea, haemorrhage
- CNS: headache, seizures, stroke, visual loss
- Skin: livedo reticularis, urticaria, purpura, erythema multiforme
Aetiologies
- Immune complex deposition e.g. Hepatitis B and PAN
- Endothelial cell infection : HIV
- ANCA mediated disease: Wegener's and microscopic polyangiitis
- T cell dependent injury: Giant cell arteritis
- Anti endothelial antibodies : Kawasaki disease and Behcet syndrome
Classification: Chapel Hill 2012
- Large vessels (LVV)
- Takayasu arteritis (TAK)
- Giant cell arteritis (GCA)
- Isolated aortitis
- Primary angiitis of CNS (PACNS)
- Medium vessel vasculitis (MVV)
- Polyarteritis nodosa (PAN)
- Kawasaki disease (KD)
- Small vessel vasculitis (SVV)
- ANCA associated vasculitis
- Microscopic polyangiitis (MPA)
- Granulomatosis with polyangiitis (Wegener) (GPA)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)
- Immune complex Small Vessel Vasculitis
- Anti-glomerular basement membrane (anti-GBM) disease
- Cryoglobulinemic vasculitis (CV)
- IgA vasculitis (Henoch-Schonlein) (IgAV)
- Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)
- Variable vessel vasculitis (VVV)
- Behcet disease (BD)
- Cogan syndrome (CS)
- Single-organ vasculitis (SOV)
- Cutaneous leukocytoclastic angiitis
- Cutaneous arteritis
- Primary central nervous system vasculitis
- Isolated aortitis
- Others
- Vasculitis associated with systemic disease
- Lupus vasculitis
- Rheumatoid vasculitis
- Sarcoid vasculitis
- Others
- Vasculitis associated with probable etiology
- Hepatitis C virus-associated cryoglobulinemic vasculitis
- Hepatitis B virus-associated vasculitis
- Syphilis-associated aortitis
- Drug-associated immune complex vasculitis
- Drug-associated ANCA-associated vasculitis
- Cancer-associated vasculitis
- Others
Investigations
- U&E FBC CXR
- Elevated ESR and CRP
- ANCA, ENA, RF, ANA, Anti dsDNA, C3, C4, Cryoglobulin
- Anticardiolipin
- Hepatitis B and C serology
- Blood cultures
- Angiography: beading of arteries with areas of constriction and dilation
- Skin biopsy
Managment
- Biopsy is important to get a reliable diagnosis especially before starting powerful immunosuppressants such as high dose steroids or cyclophosphamide
- Significant mortality from renal, lung and vascular disease depending on the type