Vasculitis Flashcards
What is vasculitis
Inflammation of blood vessels
Prolonged inflammation can lead to thrombosis, ischemia, organ infarction necrosis and even death
Which blood vessels can vasculitis
ANY
Arteries, arterioles, veins, venules or capillaries
What causes secondary vasculitis
May be triggered by an infection, drugs, a toxin or may occur as part of another inflammatory disorder or cancer
How do you classify vasculitis
Based on which type of blood vessel is affected
Small, medium or large
How does vasculitis present
Very variable - dependant in which vessels are affected
Systemic symptoms such as fever, malaise, weight loss and fatigue are common to all types
Can cause thrombosis and infarction as the inflammation of the blood vessel narrows the lumen
What does Giant cell arteritis typically cause
Temporal arteritis
May affect aorta and other large vessels
What is seen in blood vessel walls in vasculitis
Granuloma formation
What are the presenting features of large vessel vasculitis
Symptoms are dependant on which arterial branches are affected
Bruit - most commonly in carotid
Blood pressure difference in extremities
Takayasu - cannot palpate peripheral pulses
GCA
Headache
Diplopia or visual loss
Claudication - jaw and tongue
What are the signs of temporal arteritis
Unilateral temporal headache
Scalp tenderness
Jaw claudication
Temporal arteries become prominent but with reduced pulsation
What is the major risk with temporal arteritis
Blindness
Due to ischaemia of the optic nerve
What investigations do you do for large vessel vasculitis
ESR, plasma viscosity and CRP - will be raised
Temporal artery biopsy - GOLD STANDARD
MR angiogram or PET CT
How is small vessel vasculitis divided
Into ANCA associated vasculitis (AAV) and ANCA negative
Describe the pathology of GPA
Granulomatous inflammation of respiratory tract, ENT system and small and medium vessels
Necrotising glomerulonephritis common
Describe the pathology of EGPA
Eosinophilic granulomatous inflammation of respiratory tract, ENT system, small and medium vessels.
Associated with asthma
Describe the pathology of MPA
Necrotising vasculitis with few immune deposits.
Necrotising glomerulonephritis very common
What are the ENT features of GPA
Sinusitis Nasal crusting Blocked nose Epistaxis Mouth ulcers Sensorineural deafness - bilateral Otitis media and deafness “Saddle nose” due to cartilage ischaemia and necrosis
What is epistaxis
Nose bleed
What are the cutaneous features of GPA and what causes them
Palpable purpura
Petichial rashes
Cutaneous ulcers
Caused by inflammation of the small blood vessels in the skin
What are the respiratory features of GPA
Pulmonary infiltrates Interstitial lung disease Cough and SOB Haemoptysis Diffuse alveolar haemorrhage Cavitating nodules on CXR
Stridor caused by tracheal inflammation and resulting subglottic stenosis
What is the key renal feature of GPA
Necrotising glomerulonephritis
Can lead to rapid renal failure
Look for blood and/or protein in urine. Any more than a trace requires further tests (red cell cast test and protein creatinine ratio)
May go onto get renal biopsy
What are the nervous system features of GPA
Mononeuritis multiplex - foot and wrist drops
Peripheral neuropathy - glove and stocking
Cranial nerve palsies
What are the ocular features of GPA
Conjunctivitis Episcleritis Scleritis - nodular Uveitis Optic nerve vasculitis Retinal artery occlusion Proptosis Retro orbital granulomas mimicking tumours
What are ANCAs
Anti-neutrophil cytoplasmic antibodies
Autoantibodies against the cytoplasm of neutrophil granulocytes
How can you detect ANCA
Immunofluorescence
Also allows you to determine the pattern and differentiate between C-ANCA and P-ANCA
What is HSP
Henoch-Schönlein purpura
Acute IgA mediated disorder
Generalized vasculitis - not ANCA associated
What blood vessels are affected by HSP
Iinvolves the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system
HSP usually has a preceding infection - true or false
TRUE
>75% have URTI, pharyngeal or GI infection Usually about 1-3 weeks before HSP
What organism most commonly causes the infection preceding HSP
Group-A strep
How does HSP present
Purpuric rash typically over buttocks and lower limbs Colicky abdominal pain Bloody diarrhoea Joint pain +/- swelling Renal involvement - 50%
How do you manage HSP
Usually self limiting and will resolve in 8 weeks
Need to check for renal involvement and treat accordingly
What are the main subtypes of large vessel vasculitis
Giant cell arteritis - seen in over 50s
Takayasu - seen in younger women
Which vessels are affected in large vessel vasculitis
Involves the aorta and branches
What are the main subtypes of medium vessel vasculitis
Kawasaki disease - seen in kids
polyarthritis nodosa
Rarest types of vasculitis
List the ANCA associated small vessel vasculitis
Microscopic polyangitis (MPA)
Granulomatosis
with polyangiitis (GPA
Previously Wegener’s
Eosinophilic Granulomatosis
with polyangiitis (EGPA)
Previously Churg Strauss
ANCA negative small vessel vasculitis is also called what
Immune complex small vessel vasculitides
Includes HSP, anti-GBM disease etc
What are the MSK features of GPA
Myalgia
Arthralgia
Arthritis
What are the cardio features of GPA
pericarditis, coronary
vasculitis myocarditis if coronary arteries are involved
What is the screening test for small vessel vasculitis
ANCA or anti-neutrophil cytoplasmic antibody
Which blood tests may be abnormal in vasculitis
FBC - Anaemia of chronic disease(normocytic, normochromic
anaemia) is a common finding
CRP/ESR/PV - usually raised due to inflammation
LFTs can be deranged if liver involvement
U&E deranged if renal involvement
Which subtypes of ANCA are seen in GPA
c-ANCA and anti-PR3
Which subtypes of ANCA are seen in EGPA and MPA
p-ANCA
Directed against myeloperoxidase (MPO)
Anti-MPO
Can ANCA be used to monitor disease activity
Yes
Titres often rise with increasing disease activity
How do you treat vasculitis
Basically immunosuppression (lifelong) with steroids and steroid sparing agents such as cyclophosphamide,
rituximab, methotrexate, azathioprine etc
Choice of agent depends on how severe the disease is - use the 5 factor score to see how many organ systems are involved
If they score 1 or more - need high dose steroids with either cyclophosphamide or rituximab
Then move to steroid sparing agent when in remission or if low severity
What prophylaxis is require for patients on
cyclophosphamide
Pneumocystis jirovecii prophylaxis with
Cotrimoxazole
