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Vasculitis (small and large) (Large cell Vasculitis (Diagnostic Criteria:…
Vasculitis (small and large)
Definition: Vasculitis is a condition that is characterized by the inflammation of small to large blood vessels. This can ultimately lead to cell necrosis and subsequent ischemia of vessels.
Their classification is dependent on the size of vessel affected, whether ANCA is involved and if the condition is primary or secondary.
Generally vasculitis is a rare condition that predominately affects women. The main causes of it are: malignancy, infection, autoimmune, drugs and being idiopathic.
There are two types of vasculitis Large cell or Small Cell
General symptoms: fever, malaise, headaches, rash, weight loss and arthritis pain
General signs: prominent vessels, bowel infarction, stroke, foot drop or arthritic signs (similar to RA)
Differential Diagnosis
Cholesterol Emboli
Malignancy
Septic Hepatitis
Large cell Vasculitis
Aetiology:
types of the condition include Giant Cell (Temporal) and Takyasu's arteritis. Arteritis is the inflammation of the big arteries in the body
Pathophysiology:
a granulomatous reaction that causes deposition in the aorta and the larger vessels. This can can happen in the carrotd artery. These granulomatous deposits build up and cause the lumen to be obstructed. Leading to a stroke
Epidemiology:
this is primarily a disease that affects people aged 50 or older. With incidence that increases with age and in women. Generally a primary disease
Symptoms:
malaise, headache and scalp tenderness
Definition:
this is a form of vasculitis that only affects the large vessels of the body. Generally the aorta and the temporal arteries are affected. This causes a characteristic set of symptoms. This is sero negative for ANCA
Signs:
jaw claudication, temporal arteries are tender and palpable, patient may present with acute blindness, stroke or MI. They patient may also present with symptoms of polymyalgia rheumatica
Diagnosis:
Gold Standard
is a
large temporal artery biopsy
as the vasculitis can involve skip lesions. The biopsy is then sent to
histology
where inflammatory infiltrates are looked for. Then do a
AION to look for visual change causes such as a white optic disc
.
Diagnostic Criteria: if the patient has 3 or more of the following symptoms:
New Headache
Temporal artery tenderness or decreased pulsation
Abnormal artery biopsy (necrotizing arthritis and infiltrates + granulomatous inflammation)
Older than 50
Treatment:
cortico steriods and life style advice. Alongside regular DEXA scans
Small Vessel Vasculitis- Look at respiratory section
Pathophysiology:
necrotising, granulomatous vasculitis that is associated with c-ANCA. Affects all organ systems but in particular the URT, kidneys, lungs and skin . If the kidneys are not involved it is classed as a limited disease
Symptoms :
patient will have increased respiratory problems, feel tired, malaise and may also have a fever.
Aetiology: there are 3 types.
Primary granulomatosis with polyangiitis (Wegner's), Eosinophilic granulomatosis with polyangitis and microscopic polyangiitis
Signs:
pulmonary haemorrhage,sinusitis otitis, glomerulonephritis , ulcers, saddle nose deformity, synovitis and pericarditis . Alongside uveitis and scleritis
Epidemiology:
: more common in men but rare in the UK . Associated with middle age and also HEP B
Diagnosis :
c-ANCA and a CXR
Prognosis:
90% dead in 2 years
Treatment :
prednisone, cyclophosphamide and infliximab