Vasculitis Flashcards
What?
Inflammation of blood vessels
What can this lead to?
Inflammation/ Ischaemia/ Necrosis of tissues that vessel supplies
Primary vasculitis?
Results from an inflammatory response that targets the vessel walls and has no known cause.
Secondary vasculitis?
May be triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder (e.g. RA or lupus) or cancer
3 main categories?
Large, medium and small vessel
What is large vessel vasculitis?
Causes granulomatous inflammation predominantly of aorta and its major branches
2 main categories of large vessel vasculitis?
Temporal (giant cell) arteritis Takayasu arteritis (TA)
Age of onset in large vessel vasculitis?
GCA - after 50
TA - before 50
Who gets takayasu arteritis?
> In East Asian countries
Young women in 2nd and 3rd decades
Females
Pathology of large vessel vasculitis?
Granulomatous infiltration of walls of large vessels
Takayasu arteritis?
Claudication
Pain in arm muscles e.g. when doing housework
Bruit – carotid artery
BP difference – between left and right side extremities -> pulseless disease
Angiogram
GCA?
Temporal arteritis Prominent temporal arteries Strong assoc. with polymyalgia rheumatica Pain in jaw when chewing food Tongue claudication - linguine artery
Features of temporal arteritis?
Unilateral acute temporal headache Scalp tenderness Temporary visual disturbances Blindness Jaw claudication
Clinical features of large cell vascultiis?
Low- grade fever Malaise Night sweats Weight loss arthralgia Fatigue Claudicant symptoms (both upper and lower limbs) Untreated -> vascular stenosis and aneurysms (reduced pulses and bruits)
Investigations for large vessel vasculitis?
ESR, CRP and plasma viscosity elevated
MR angiography –> thickened vessels & vessel stenosis
PET CT – if inflammation present
Investigations for GCA?
TEMPORAL ARTERY BIOPSY
- Skip lesions occur so biopsy may be false neg
Ultrasound
- 24- 48 hrs after start steroids
Treatment for large vessel vasculitis?
Corticosteroids (prednisolone 40- 60mg)
Methotrexate and azathioprine (may be added)
Immunosuppressives in TA?
Leflunamide, methotrexate
Immunosuppressives in relapsing or resistant GCA?
Tocilizumab – IL-6 blocking agent
How long is GCA treatment?
18 months to 2 years
4 types of ANCA associated vasculitis?
Granulomatosis with polyangitis
Microscopic polyangitis
Renal limited vasculitis
Eosinophilic granulomatosis with polyangitis
Who gets granulomatosis with polyangitis (GPA/ wagerers)?
Northern Europe
Males >
35- 55 years
Symptoms of GPA?
Constitutional symptoms and arthralgia ENT symptoms Respiratory symptoms Cutaneous Renal
ENT symptoms of GPA?
Nose bleeds Deafness Recurrent sinusitis Nasal crusting (over time can be collapse of nose) Epistaxis Mouth ulcers “Saddle nose” Sub- glottic inflammation (trouble breathing, inflammation) Proptosis
Resp symptoms of GPA?
Haemoptysis Cavitating lesions on x-ray Cough Pulmonary infiltrates Diffuse alveolar heamorrhages
Cutaneous symptoms of GPA?
Palpable purpura
Cutaneous ulcers
Renal symptoms of GPA?
Necrotizing glomerulonephritis
What is GPA associated with?
cANCA and PR3
EGPA?
Characterized by late onset asthma, rhinitis and raised peripheral blood eosinophil count
Mono neuritis multiplex
Complications of microscopic polyangitis?
Glomerulonephritis
Investigations for ANCA associated vasculitis?
ESR, PV and CRP raised
Anaemic of chronic disease common
U + E (renal involvement?)
Anto- neutrophil cytoplasmic antibody (ANCA)
Urinalysis (looking for renal vasculitis)
CXR
Biopsy (of affected area)
Management of ANCA assoc vasculitis?
IV steroids and cyclophosphamide
Henoch Schonlein purpura?
Acute immunoglobulin A (IgA) mediated disorder
Generalized vasculitis involving small vessels of skin, GI tract, kidneys, joints & rarely lungs and CNS
Group A streptococcus (most common)
Who gets Henoch Schonlein purpura?
Children (2-11)
History of upper resp tract infection (few weeks)
Rare in infants
Symptoms of Henoch Schonlein purpura?
Purpuric rash over buttocks and lower limbs Abdominal pain (colicky) Vomiting Joint pain +/- swelling Bloody diarrhea Renal involvement
Management of Henoch Schonlein purpura?
Self- limiting – settles over weeks to months
Investigations for vasculitis?
Urine dipstick
FBC, Liver and renal profile, inflammatory markers
ANCA and specific antibodies, connective tissue disease screen, compliment levels
Imaging investigations – Chest x-ray and/or CT scan
Nerve conduction tests (neurological symptoms?)
Tissue biopsy (most definitive test) - GOLD
ANCA?
Anti- neutrophil cytoplasmic antibodies
Autoantibodies against antigens in cytoplasms of neutrophil granulocytes
ANCA in GPA?
cANCA
ANCA in EGPA?
pANCA
ANCA in MPA?
pANCA
