Vasculitis Flashcards
What are the kinds of vasculitis?
Primary - no underlying cause
Secondary - result of drugs, bugs or other autoimmune diseases eg SLE.
Giant cell arteritis ?
Large vessel arteritis
High inflammatory makers
New headache
Temporal artery abnormality
Visual symptoms
More common in elderly
Takayasu arteritis?
Young women (under age 40)
Systemically unwell - myalgia or arthralgia
Claudication - as affects large vessels
Feeble or unequal pulses
Possible aortic murmur
polyarteritis nodosa?
Generally age 40-50. More common in men.
Systemic - Weight loss
Neuropathy
Gastroenteritis all - bowel ischaemia, infarction pancreatitis
Testicular
Leg ulcer
Renal involvement - hypertension’s
ANCA test negative
Kawasaki disease?
Childhood (under age of 5)
Caused by infectious trigger?
Small and medium vessels affected
Fever
Bilateral conjunctival congestion
Oral mucosa involvement - strawberry tongue, redding of lips/lining of mouth
Anchor negative
Oedema of hands and feet, red palms and soles
Skin rash on trunk
Cervical lymphadenopathy
Wegeners granulmatosis/ granulomatosis with polyangitis?
Granulomata and vasculitis
ENT involvement
Pulmonary-renal syndrome
Associated with proteinase 3 ANCA
Generally fatal if untreated
Microscopic polyangitis ?
No granulomata
Predominantly kidney affected
Systemic illness
Renal involvement
Myeloperosidase ANCA positive
Churg-Strauss/ eosinophilic granulomatosis?
Resistant asthma (late onset)
Peripheral eosinophilia
Neuropathy
Cardiac involvement
Nerve involvement, pulmonary infiltration
Associated with myeloperoxidase ANCA
What is the differential diagnosis of vasculitis?
Bacterial endocarditis
Cancer (paraneoplastic)
SLE
Atrial myxoma
Choestrol emboli
Calciphylaxis
Bloods tests for vasculitis ?
FBC - neutrophils, eosinophilia
ESR raised
Biochem - assess organ involvement
CRP raised
Complement levels
RF
Protein electrophoresis
Serum immunglobulins
Cryoglobulins
ANCA
What is ANCA test?
Detects Antineutrophil cytoplasmic antibody in blood - positive in 3 key diseases granulomatosis with polyangitis (Wegener granulomatosis), eosinophilia granulomatosis with polyangitis (churg-Strauss), microscopic polyangitis (MPA).
Generally seen in small vessel arteritis.
Indirect immunofluorescence - cytoplasmic, perinuclear
ELISA -proteinase 3, myeloperosidase
Urine analysis for vasculitis?
Blood
Protein
Casts
Histological biopsy?
Yield variable
Renal best - almost always positive
Arterial biopsy can be done
Imaging for vasculitis?
Artery specific imaging for large an medium vessel disease - angiography (shows micro-aneurisms), magnetic resonance, positron emission tomography
Tissues specific imaging for small vessel vasculitis
Treatment for large vessels disease?
Glucocorticoids
Immunosuppressive agents
PAN, MPA, WG, CSS treatments?
Localised = Methotrexate And Steriods (high-dose)
Generalised = cyclophosphamide and steroids (high-dose)
If ANCA positive = rituximab
Intravenous cyclophosphamide - can cause infertility, and bladder irritability (increases risk of malignancy)
Maintenance = Aziathioprine, methotrexate, MMF, glucocorticoids (low-dose)
Outcome for vasculitis?
Night mortality without treatment
60-75% survive 10yr with treatment
Henoch- schonlein purpura?
Small-vessel vasculitis caused by immune complex deposition after an infectious trigger.
Disease of children and young adults
Clinical features - purpura over buttocks and lower legs, abdominal pain, GI bleeding, arthralgia, nephritis up to 4 weeks after other symptoms.
Behçet’s disease?
Targets small arteries and venules
More common in ‘silk route’ countries
Associated with HLA-B51
Oral ulcers in all (deep)
Genital ulcers are common
Skin lesions
Eye involvement
Neurological involvement
Recent thrombosis
Renal involvement is rare
Pathergy test
Treatment for behcet’s ?
Oral ulcers - topical glucocorticoid (soluble prednisone mouthwash)
Erythema nodosum (skin lesion) - colchicine for skin and arthralgia
Oral and genital ulceration - thalidomide is teratogenic and neurotoxic - not used anymore
Uveitis and neurological disease - glucocorticoids and immunosuppressants
Still’s disease?
Rare systemic auto-inflammatory disease
Intermittent fever
Rash
Arthralgia
Splenomegaly, hepatomegaly, lymphadenopathy
Acute phase response (ferritin high)
CRP, RF, ANA negative
Treatment for Still’s?
Glucocorticoids
Methotrexate, axiothiaprine or MMF
Anakinra
Investigations and treatment for henoch-schonlein?
Biopsy of affected tissue shows a vasculitis with IgA deposits in vessel wall.
Usually self limiting
Is severe - glucocorticoids, immunosuppressive therapy
