Vasculitides Flashcards

1
Q

Vasculitis types

A

Large - giant cell arteritis, Takayasu’s arteritis
Medium - Polyarteritis nodosa, Kawasaki disease
Small - microscopic polyangiitis, Wegener’s granulomatosis, Churg-Strauss syndrome, Goodpasture’s, Henoch-Schonlein purpura

Variable vessel vasculitis - Behçet’s, Cogan’s

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2
Q

What is GCA

A

Giant cell arteritis = temporal arteritis

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3
Q

GCA presentation

A

Headache (temporal artery/scalp tenderness)
Tongue/jaw claudication
Amaurosis fugax (temp blindness)
>55 yrs

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4
Q

GCA associations

A

Polymyalgia rheumatica in 50%

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5
Q

GCA investigations

A

ESR + CRP very raised, dec Hb

Vision testing, damage often irreversible

Temporal artery biopsy (biopsy sometimes negative as occasionally skip lesions)
FDG-PET

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6
Q

GCA management

A

Prednisolone 60mg/d oral immediately, IV methylpred if sight loss occurring
Typically 2yr course induces remission, reduce pred once ESR decreased (main cause of mort/morb is steroid use so balance risk)
Use PPI/bisphosphonates/calcium with colecalciferol/aspirin if needed

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7
Q

What is Takayasu’s arteritis

A

Granulomatous inflammation of aorta and major branches causing systemic effects from stenosis/thrombosis/aneurysm of aorta

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8
Q

Takayasu’s arteritis presentation

A

Rare outside of Japan
20-40yr old women typically
Symptoms depend on arteries involved, can be cerebral/eye/upper limb

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9
Q

Takayasu’s arteritis management

A

BP control

Very good prognosis, 1mg/kg/day prednisolone
Methotrexate/cyclophosphamide in resistant disease

Surgery for critical stenosis

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10
Q

What is polyarteritis nodosa

A

Necrotising vasculitis that causes aneurysms + thrombosis in medium-sized arteries, symptoms depend on infarcted arteries

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11
Q

Polyarteritis nodosa presentation

A

Rare in UK and can be associated with Hep B
Rash, punched out ulcers
Renal/cardiac/GI/GU issues

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12
Q

Polyarteritis nodosa investigations

A

ANCA -ve
Inc WCC, ESR + CRP

Renal/mesenteric angiography or renal biopsy can be diagnostic

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13
Q

Polyarteritis nodosa management

A

Control BP

Steroids for mild cases, steroid sparing if more severe

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14
Q

What is Kawasaki disease

A

Febrile vasculitis causing coronary aneurysms

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15
Q

Kawasaki disease presentation

A

Typically 18-24 mths
3 phases:
Acute febrile for 1-2 wks
Subacute lasts 2-4 wks after fever and CA aneurysms develop, thrombocytosis
Convalescent wks 6-12, resolution of signs + inflammatory markers

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16
Q

Kawasaki disease management

A

IVIg 2g/kg single dose within 10d of symptom onset to decrease new CA aneurysms
IVIG+pred if unresponsive to initial dose
Aspirin
Good prognosis, mortality ~1%

17
Q

Kawasaki disease investigations

A

Raised ESR/CRP
LFTs
A1-antitrypsin

Platelets

MR angiography/echo for aneurysms

18
Q

What is Microscopic polyangiitis

A

Necrotising vasculitis affecting small + medium sized vessels

19
Q

Microscopic polyangiitis presentation

A

Typically rapidly progressing glomerulonephritis

Pulmonary haemorrhage in ~30%

20
Q

Microscopic polyangiitis investigations

A

p-ANCA +ve

21
Q

Microscopic polyangiitis management

A

Steroids acutely
Methotrexate if resistant
Methotrexate/rituximab/azathioprine for maintenance

22
Q

What is hypocomplementic urticarial vasculitis

A

Lupus-like illness with urticaria + Abs to C1q

23
Q

What is polymyalgia rheumatica

A

Unknown pathogenesis, similar features to GCA but not a vasculitis

24
Q

Polymyalgia rheumatica presentation

A

> 50yrs
Onset <2wks bilateral aching, tenderness + morning stiffness, especially proximal limb muscles
Weight loss, anorexia, depression

25
Q

Polymyalgia rheumatica investigations

A

Raised CRP, sometimes ESR normal

Creatine kinase normal (distinguish from myositis)

26
Q

Polymyalgia rheumatica management

A

Prednisolone 15mg/d PO (should have dramatic response within a wk, if not then consider other diagnosis)
Most need steroids for >2yrs so give bone protection
+ Methotrexate for pts with relapse/prolonged therapy

27
Q

What is Behçet’s disease

A

Systemic inflammatory disorder of unknown cause

28
Q

Behçet’s disease presentation

A

Associated with HLA-B5, common along old Silk Road from Mediterranean to China

Recurrent oral + genital ulceration is big clue

Uveitis, skin lesions
Arthritis
Vasculitis
Myo/Pericarditis
Colitis
29
Q

Behçet’s disease diagnosis

A

Mainly clinical

Pathergy’s test (needle prick leads to papule formation <48 hrs)

30
Q

Behçet’s disease management

A

Steroids
Azathioprine/cyclophosphamide for systemic disease

Infliximab in ocular disease unresponsive to topical steroids

Colchicine for orogenital ulceration

31
Q

Paget’s disease clinical features

A

Isolated raised ALP

Pains along centre of body e.g. spine, pelvis