Vasculitis Flashcards

1
Q

What types of vasculitis affect the small vessels?

A

Granulomatosis with polyangitis (GPA)
Eosinophilic granulamotosis with polyangitis (EGPA; Churg-Strauss syndrome)
Microscopic polyangitis
Drug-induced vasculitis

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2
Q

What types of vasculitis affect the medium vessels?

A

Polyarteritis nodosa (PAN)
Kawasaki disease
Behcet’s Disease

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3
Q

What type of vasculitis affects the large vessels?

A

Giant cell arteritis (GCA) and polymylagia rheumatica

Takayasu arteritis

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4
Q

What organs are affected in GPA?

A

Upper and lower respiratory tract

Kidneys

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5
Q

What features are present in GPA?

A

ANCA-associated vasculitis features

Features specific to GPA:
Epistaxis
Nasal crusting
Saddle-shape nose deformity
Eye involvement e.g. proptosis
Cranial nerve lesions
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6
Q

What investigations findings suggest a diagnosis of GPA?

A

cANCA +ve

CXR:
May display lung cavities

Urine dip:
Blood and protein in urine would suggest glomerulonephritis

Renal biopsy:
Epithelial crescents in Bowman’s capsule

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7
Q

What is the management for the ANCA-associated vasculitis conditions?

A
Steroids:
Oral prednisolone
IV hydrocortisone
Nasal spray
Inhaled if lung involvement e.g. EPGA
Immunosuppressants:
Cyclophosphamide
Methotrexate
Azathioprine
Rituximab and other monoclonal antibodies

Plasma exchange

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8
Q

What are the features of EGPA?

A

ANCA-associated vasculitis features

Features specific to EGPA:
Asthma
Paranasal sinusitis
Multineuritis multiplex
Eosinophilia of >10% of peripheral blood
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9
Q

What investigations findings suggest a diagnosis of EGPA?

A

pANCA +ve

FBC:
Elevated eosinophil level

CXR:
Lung cavities

Urine dip:
Blood and protein in urine would suggest glomerulonephritis

Renal biopsy:
Epithelial crescents in Bowman’s capsule

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10
Q

What common features do ANCA-associated vasculitis conditions have?

A

Renal impairment:
Rapid progressive glomerulonephritis - raised creatinine, haematuria, proteinuria

Respiratory symptoms:
SOB
Haemoptysis

ENT:
Sinusitis

Skin:
Vasculitis rash

Systemic symptoms:
Fatigue
Weight loss
Fever

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11
Q

What other conditions are associated with pANCA antibody?

A

Ulcerative colitis (70%)
Primary sclerosing cholangitis (70%)
Anti-GBM disease (25%)
Crohn’s disease (20%)

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12
Q

What features are present in microscopic polyangitis?

A

ANCA-associated vasculitis features

Mainly causes renal failure, SOB and haemoptysis

pANCA +ve in 50-75%
cANCA +ve in 40%

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13
Q

What condition is PAN associated with?

A

Hepatitis B

Can also occur in hep C and HIV

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14
Q

What are the features of PAN?

A
Livedo reticularis
Fever, malaise, weight loss
Arthralgia
Hypertension
Mononeuritis multiplex
Sensorimotor polyneuropathy
Testicular pain
Haematuria
Renal failure

pANCA in 20%
Hep B serology +ve in 30%

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15
Q

What are the features of Kawasaki disease?

A

Persistent fever lasting >5 days and is resistant to antipyretics
Conjunctival injection in eye
Bright red, cracked lips
Strawberry tongue
Cervical lymphadenopathy
Erythema and desquamation (skin peeling) of palms and soles
Erythematous rash

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16
Q

What is the management for Kawasaki disease?

A

High-dose aspirin
IV immunoglobulin

Echo to screen for coronary artery aneurysms

17
Q

What is the key complication that can occur in Kawasaki disease?

A

Coronary artery aneurysms

18
Q

What are the features of Behcet’s disease?

A

Classic triad:
Oral ulcers
Genital ulcers
Anterior uveitis

Thrombophlebitis and DVT
Erythema nodosum
Arthritis
Neurological involvement e.g aseptic meningitis
GI issue - abdominal pain, diarrhoea, colitis

19
Q

What investigation is carried out in Behcet’s disease?

A

Pathergy test

20
Q

What is the management for Behcet’s disease?

A
Topical steroids for mouth ulcers (e.g. soluble betamethasone tablets)
Oral prednisolone
Colchicine
Lidocaine ointment for genital ulcers
Immunosuppressants
Biological therapy
21
Q

What are the features of Takayasu’s arteritis?

A

Absent limb pulse due to occlusion of aorta
Systemic features of vasculitis e.g. malaise, headache
Unequel BP in upper limbs
Carotid bruits
Intermittent claudication
Aortic regurgitation (in 20%)

22
Q

What condition is Takayasu’s arteritis associated with?

A

Renal artery stenosis

23
Q

What is the management for Takayasu’s arteritis?

A

Steroids