Vasculitis Flashcards
What is vasculitis?
Inflammation of the blood vessels
- a response to an insult with many causes (primary and secondary)
What are the secondary causes of vasculitis?
Infection Underlying disease - cancer - autoimmune disease such as rheumatoid arthritis, SLE, or IBD Cold injury Drugs
What are the key examples of infection causing vasculitis?
Meningococcal septicaemia
Streptococcus
Hepatitis B
Hepatitis C
How can a cold injury cause vasculitis?
Frost-bite
- can cause local vascular injury and tissue necrosis
What is cryoglobulinaemia?
The cold causes autoantibodies to be activated, and then attack, clump and destroy red blood cells.
This blocks and irriates the vessels (vasculitis)
The peripheral (coldest parts of the body) are affected worst.
Blockage of the blood vessels causes gangrene
Which drugs can causes vasculitis?
Anti-thyroid drugs (carbimazole) Minocyclin Hydralazine Penicillamine Antibiotics (penicillin/sulphonamides) Anticonvulsants
What are the general features of primary vasculitis?
Systemic inflammation - fever, malasie, weight loss, night sweats, algia (muscles/joints) Large vessels affected - end organ ischaemia or infarction Medium vessels affected - localised ischaemia or infarction Small vessels affected - organ specific inflammation
What are the two types of large vessel involvement in vasculitis?
Giant cell arteritis
- very common in the elderly
Takayasu
- very rare
What are the two types of medium vessel involvement in vasculitis?
Polyarteritis nodosa Kawasaki disease (only in children)
What are the two types of small vessel involvement in vasculitis?
Immune complex mediated vasculitis - HSP - most common type of vasculitis - cryoglobulinaemia ANCA associated vasculitis - microscopic polyangiitis - granulomatosis with polyangiitis - eosinophilic granulomatosis with polyangiitis
What are the clinical features of giant cell arteritis?
Systemic features Fatigue Headaches Jaw claudication Scalp tenderness Confusion Stroke Sensorineural deafness Depression Depends on the artery affected - ophthalmic artery (sudden painless onset blindness) - lingual artery (tingling in unilateral tongue)
What tests can be run to diagnose giant cell arteritis?
Very high ESR
Biopsy
What is polymyalgia rheumatica?
Considered to be a related disorder to giant cell arteritis.
A syndrome of myalgia, typically in the neck, shoulders, upper arms and hips
What are the symptoms of polymyalgia rheumatica?
Pain and stiffness in the muscles of the neck, shoulder girdle and pelvic girdle
(proximal muscles - rheumatological pattern)
No weakness
Name a test to diagnose polymyalgia rheumatica.
High ESR
Improves with steroids
What is Takayasu’s arteritis?
A rare, systemic, inflammatory large-vessel vasculitis
- granulomatous inflammation of the aorta and it’s major branches
What is the epidemiology of Takayasu’s arteritis?
Young (15-25yrs)
Women
What are the clinical features of Takayasu’s arteritis?
Systemic features (early) Vascular insufficiency features (late disease) - bruitis - absent, reduced pulses - claudication - ischaemic heart disease, heart failure or pulmonary hypertension - headaches - sudden, painless blindness - BP variable
How is Takayasu’s arteritis diagnosed?
Imaging angiography
MR angiography
CT scan
Shows narrowing of the aorta and larger arteries adjacent to the aorta
What are the clinical features of Polyarteritis nodosa?
Medium sized vessel vasculitis - systemic features - malasise - myalgia/arthralgia - organ infarction E.g. Kidney - renal artery stenosis - renal aneurysms - hypertension
Which organs are typically affected by polyarteritis nodosa?
Gut Brain - stroke Heart - MI/angina Liver Skin - gangrene, purpura, nodules PNS - pain over dermatome, loss of sensory or motor function Testis - pain or infarct Limbs - claudication Kidney
What is Kawasaki disease?
A disease of children and babies
Aneurysms forms in medium arteries
Which specific arteries does Kawasaki disease affect?
Coronary arteries - MI
Axillary arteries
Iliac arteries
Popliteal arteries
What are the early features of Kawasaki disease?
High fever Arthritis Myocarditis Red palms/soles Desqaumation/Rash Straeberry tongue Cervical lymphadenopathy Thrombocytosis Miserable Mucositis Conjunctivitis
What is HSP?
The most common cause of immune complex small vessel vasculitis in childhood
Possibly has an infective cause
IgA deposits in the gut, skin and synovial lesions
What is the diagnostic triad of HSP?
Palpable purpura
- covers the extensor surfaces, starting peripherally
Abdominal pain
Arthritis
How do IgA deposits affect the gut, kidney, joints and skin?
Joints - pattern of joint involvement matches the skin - starts peripherally and works proximally Kidney - nephritic syndrome Skin - palpable purpura Gut - causes abdominal pain
What is ANCA positive vasculitis?
Anti-neutrophil cytoplasmic antibodie positive vasculitis
- cytoplasm of cells stain green on ANCA test
What are the two types of ANCA vasculitis?
cANCA - specific for granulomatous polyangiits pANCA (MPO positive) - microscopic polyangiitis - crescentic glomerulonephritis - eosinophilic granulomatous polyangiitis pANCA (MPO negative) - IBD - RA - Sclerosing cholangitis
Which blood vessels does granulomatous polyangiits normally affect?
Nose Sinuses Ears Lungs Kidneys Although other organs can be affected
What are the symptoms of granulomatous polyangiitis?
Systemic symptoms Malaise Joint pain Persistent ear infections and reduced hearing Sinusitis Nosebleeds Crusts around the nose Chest pain, cough and SOB Coughing up blood Blood in the urine
What are the most commonly affected blood vessels in eosinophilic granulomatous angiitis?
Lungs and skin are most common Can affect all other organs - heart - kidneys - nerves - bowel
What are the common symptoms of eosinophilic granulomatous angiitis?
Asthma Eosinophilic infiltrates (transient pulmonary infiltrates) Eosinophilia - everywhere Eosinophil rich granulomata Peripheral neuropathy
What are the five most common clinical manifestations of microscopic polyangiitis?
Kidney glomerulonephritis Weight loss Skin lesions Peripheral neuropathy, mononeuritis multiplex Fevers
What tests can you do to assess a patient for inflammation?
Temperature chart
Height and weight
ESR and CRP
FBC
What vessel specific tests should you run on a person with suspected vasculitis?
Blood pressure in all four limbs Examine the vascular tress - pulses - feel for aneurysms Medium and large angiography/MRA Small - organ specific tests - US abdomen, MRI gut, endoscopy, biopsy - BP urianlysis, US kidneys, perfusion scan, biopsy - echo, ECG and muscle enzymes - US liver, LFTs, biopsy - image the CNS, LP - nerve conduction studies CXR, CT chest, pulmonary function tests, biopsy - skin biopsy
What disease specific tests should you run on a person with suspected vasculitis?
ANCA
What is the treatment for vasculitis?
Corticosteroids
Other immunosuppression
What is the most common vasculitis in children?
Henoch Schonlein Purpura
What are the symptoms associated with Henoch Schonlein Purpura?
Palpable purpura
Abdominal pain
Arthritis
Which organ requires the most monitoring in HSP?
Kidney
- BP and urinalysis
In which non-vasculitis conditions can ANCA be positive?
IBD
SLE
pANCE and MPO negative typically
What is the most common cause of vasculitis?
Infection
