Vasculitis Flashcards
What are the large vessel vasculitides?
Giant cell arteritis (GCA)
Takayasu arteritis
What is giant cell arteritis (GCA)?
Large vessel vasculitis causing skip lesions of granulomatous inflammation causing vessel wall thickening narrowing the lumen and causing distal ischaemia
How does giant cell arteritis (GCA) present?
Over 50s
Headache
Scalp tenderness
Prominent, tender temporal artery
Painless visual disturbances
Jaw claudication
What condition is giant cell arteritis (GCA) associated with and how does it present?
Polymyalgia rheumatica
Proximal muscle pain and stiffness
What blood test would you order in suspected giant cell arteritis (GCA) and what would it show?
Inflammatory markers
Raised
What is the diagnostic test for giant cell arteritis (GCA)? What do you do if it is negative but there is a strong clinical suspicion of GCA?
Temporal artery biopsy showing granulomatous inflammation
Treat as giant cell arteritis (GCA). Biopsy has high false negative rate due to skip lesion pattern
How is giant cell arteritis (GCA) treated?
Immediate prednisolone
- 40mg
- 60mg if visual symptoms
What can untreated giant cell arteritis (GCA) progress to and why?
Irreversible blindness from optic nerve ischaemia
How does Takayasu arteritis present?
Vasculitis in Asian females under 40
What are the ANCA-positive small vessel vasculitides?
Granulomatosis with polyangiitis (GPA)
Eosinophilic granulomatosis with polyangiitis (EGPA)
Microscopic polyangiitis
What are the ANCA-negative small vessel vasculitides?
Henoch-Schloein purpura (HSP)
What is granulomatosis with polyangiitis (GPA)?
Small vessel ANCA associated vasculitis causing ENT symptoms and kidney damage
What immunological markers are associated with granulomatosis with polyangiitis (GPA)
c-ANCA
PR3
How does granulomatosis with polyangiitis (GPA) present?
Constitutional symptoms
ENT symptoms (saddle nose, epistaxis, sinusitis, heating loss etc.)
Cough (haemoptysis)
Vasculitic rash
Mononeuritis multiplex
How does c-ANCA appear under immunofluorsence
Cytoplasmic-anti-neutrophil-cytoplasmic antibody
Stains around the cytoplasm
What is eosinophilic granulomatosis with polyangiitis (EGPA)?
Small vessel vasculitis causing asthma and eosinophilia
How does eosinophilic granulomatosis with polyangiitis (EGPA) present?
Constitutional symptoms
Asthma
Vasculitic rash
Mononeuritis multiplex
What immunological markers are associated with eosinophilic granulomatosis with polyangiitis (EGPA)?
p-ANCA
MPO
How are the ANCA-associated small vessel vasculitides treated?
Depends on severity
Just respiratory involvement
- methotrexate and steroids
Organ dysfunction/failure
- cyclophosphamide and steroids
- and plasma exchange if creatinine >50
- then further DMARDs
Refractory
- IV immunoglobulins
What is Henoch-Schonein purpura (HSP)?
IgA mediated small vessel vasculitis commonly affecting children
How does Henoch-Schonein purpura (HSP) present?
In children
Constitutional symptoms
Abdominal pain and bloody diarrhoea
Sore throat/cough
Arthralgia
What test needs to be done in suspected small vessel vasculitis and what are you looking for in this test?
Urinalysis
Proteinuria/haematuria which suggests kidney damage
How is Henoch-Schonein purpura (HSP) treated?
Self limiting condition so no management required
May relapse
