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MSK Diseases > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What are the kinds of vasculitis?

A

Primary - no underlying cause
Secondary - result of drugs, bugs or other autoimmune diseases eg SLE.

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2
Q

Giant cell arteritis ?

A

Large vessel arteritis
High inflammatory makers
New headache
Temporal artery abnormality
Visual symptoms
More common in elderly

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3
Q

Takayasu arteritis?

A

Young women (under age 40)
Systemically unwell - myalgia or arthralgia
Claudication - as affects large vessels
Feeble or unequal pulses
Possible aortic murmur

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4
Q

polyarteritis nodosa?

A

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

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5
Q

Kawasaki disease?

A

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

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6
Q

Wegeners granulmatosis/ granulomatosis with polyangitis?

A

Granulomata and vasculitis
ENT involvement
Pulmonary-renal syndrome
Associated with proteinase 3 ANCA
Generally fatal if untreated

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7
Q

Microscopic polyangitis ?

A

No granulomata
Predominantly kidney affected
Systemic illness
Renal involvement
Myeloperosidase ANCA positive

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8
Q

Churg-Strauss/ eosinophilic granulomatosis?

A

Resistant asthma (late onset)
Peripheral eosinophilia
Neuropathy
Cardiac involvement
Nerve involvement, pulmonary infiltration
Associated with myeloperoxidase ANCA

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9
Q

What is the differential diagnosis of vasculitis?

A

Bacterial endocarditis
Cancer (paraneoplastic)
SLE
Atrial myxoma
Choestrol emboli
Calciphylaxis

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10
Q

Bloods tests for vasculitis ?

A

FBC - neutrophils, eosinophilia
ESR raised
Biochem - assess organ involvement
CRP raised
Complement levels
RF
Protein electrophoresis
Serum immunglobulins
Cryoglobulins
ANCA

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11
Q

What is ANCA test?

A

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

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12
Q

Urine analysis for vasculitis?

A

Blood
Protein
Casts

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13
Q

Histological biopsy?

A

Yield variable
Renal best - almost always positive
Arterial biopsy can be done

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14
Q

Imaging for vasculitis?

A

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

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15
Q

Treatment for large vessels disease?

A

Glucocorticoids
Immunosuppressive agents

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16
Q

PAN, MPA, WG, CSS treatments?

A

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)

17
Q

Outcome for vasculitis?

A

Night mortality without treatment
60-75% survive 10yr with treatment

18
Q

Henoch- schonlein purpura?

A

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.

19
Q

Behçet’s disease?

A

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

20
Q

Treatment for behcet’s ?

A

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

21
Q

Still’s disease?

A

Rare systemic auto-inflammatory disease
Intermittent fever
Rash
Arthralgia
Splenomegaly, hepatomegaly, lymphadenopathy
Acute phase response (ferritin high)
CRP, RF, ANA negative

22
Q

Treatment for Still’s?

A

Glucocorticoids
Methotrexate, axiothiaprine or MMF
Anakinra

23
Q

Investigations and treatment for henoch-schonlein?

A

Biopsy of affected tissue shows a vasculitis with IgA deposits in vessel wall.

Usually self limiting
Is severe - glucocorticoids, immunosuppressive therapy

MSK Diseases (19 decks)

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