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VASCULITIS Flashcards

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1
Q

What is vasculitis?

A

Inflammation of the blood vessels

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2
Q

Chapel Hill consensus definition of vasculitis?

A

Large vessel - Takayasu + GCA
Medium - PAN + Kawasaki
Small: ANCA-associated and immune-complex-associated

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3
Q

ANCA-associated vasculitis?

A

Microscopic polyangitis
Granulomatosis with polyangitis
Eosinophilic granulomatosis with polyangitis

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4
Q

Immune-complex-associated vasculitis?

A

Anti-GBM disease
Cryoglobulinaemic vasculitis
IgA vasculitis/HSP
Hypocomplementemic urticaria vasculitis (anti-C1q)

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5
Q

Examples of medium vessel vasculitis?

A

Polyarteritis nodosa
Kawasaki disease

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6
Q

Examples of large vessel vasculitis?

A

Giant cell arthritis
Takayasu’s arteritis

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7
Q

What is granulomatosis with polyangiitis also known as?

A

Wegeners granulomatosis

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8
Q

What is Eosinophilic granulomatosis with polyangiitis also known as?

A

Churg-Strauss syndrome

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9
Q

What is anti-glomerular basement membrane disease also known as?

A

Goodpastures syndrome

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10
Q

Which ANCA type is associated with granulomatosis with polyangiitis?

A

cANCA

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11
Q

Which ANCA type is associated with Eosinophilic granulomatosis with polyangiitis?

A

pANCA

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12
Q

Most commonly targeted protein in granulomatosis with polyangiitis?

A

Serine proteinase 3 (PR3)

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13
Q

Most commonly targeted protein in Eosinophilic granulomatosis with polyangiitis?

A

MPO

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14
Q

Conditions associated with cANCA?

A

Granulomatosis with polyangiiitis
40% of microscopic polyangiitis

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15
Q

Conditions associated with pANCA?

A

75% of microscopic polyangiitis
50% of Eosinophilic granulomatosis with polyangiitis
25% of granulomatosis with polyangiitis
70% of UC
70% of PSC
25% of anti-GBM disease
20% of Crohn’s disease

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16
Q

Features of granulomatosis with polyangiitis?

A
  • upper respiratory tract: epistaxis, chronic sinusitis, nasal crusting, saddle-shape nose deformity
  • lower respiratory tract: dyspnoea, haemoptysis, cough, wheeze, cavitating lesions
  • rapidly progressive glomerulonephritis (pauci-immune)

also: vasculitic rash, eye involvement (e.g. proptosis), ear involvement (e.g. hearing loss), cranial nerve lesions, constitutional symptoms

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17
Q

Why does granulomatosis with polyangiitis cause a saddle nose deformity?

A

As it causes progressive loss of the cartilage in the nose

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18
Q

Investigtaions for granulomatosis with polyangiitis?

A

Urinalysis
FBC - anaemic, leukocytosis and thrombocytosis, raised CRP and ESR
ANCA antibodies - cANCA positive in >90% and pANCA positive in 25%
CXR - cavitating lesions, nodules, infiltrates
Renal biopsy - epithelial crescents in bowman’s capsule

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19
Q

Management of granulomatosis with polyangiitis?

A

steroids
cyclophosphamide (90% response)
plasma exchange

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20
Q

Prognosis of Granulomatosis with polyangiitis?

A

Median survival is 8-9 years

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21
Q

Clinical presentation of Eosinophilic granulomatosis with polyangiitis?

A

Severe, adult-onset asthma
Paranasal sinusitis
Mononeuritis multiplex
Vasculitis rash
Muscle and joint pain

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22
Q

Investigtaions for Eosinophilic granulomatosis with polyangiitis?

A

FBC - blood eosinophilia (>10%), anaemia, raised CRP and ESR
Serum ANCA - pANCA positive

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23
Q

Investigtaions for microscopic polyangitis?

A

ANCA - 75% have pANCA And 40% have cANCA
Renal biopsy - shows necrotising glomerulonephritis

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24
Q

Presentation of microscopic polyangiitis?

A

Renal failure due to glomerulonephritis
Vasculitis rash
Diffuse alveolar haemorrhage = haemoptysis
Mononeuritis multiplex

And constitutional symptoms

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25
What is a vasculitic rash?
Palpable purpura
26
What is Henoch-schonlein purpura?
An IgA mediated small vessel vasculitis Inflammation occurs due to IgA deposits in blood vessels
27
Who is HSP usually seen in?
Children under 10 following an infection (usually a viral RTI)
28
Presentation of HSP?
Palpable purpuric rash over buttocks and extensor surfaces of arms and legs - in 100%! Abdominal pain that is severe and colicky Polyarthritis Features of IgA nephropathy e.g. haematuria or renal failure
29
Investigtaions for HSP?
Urinalysis - check for haematoproteinuria BP - may be elevated in nephrotic syndrome FBC ESR&CRP raised U&Es - elevated cr if renal involvement Skin or renal biopsy can be performed for immunofluroresnece of IgA/C3 but this is rarely done!
30
Management of HSP?
Supportive & Analgesia for arthralgia
31
Prognosis of HSP?
Usually excellent, self-;limiting (esp in children without renal involvement) 1/3rd of pt have a relapse
32
Monitoring following HSP?
Bp and urinalysis to detect progressive renal involvement
33
What is goodpastures syndrome?
Anti-glomerular basement membrane disease A rare type of small-vessel vasculitis
34
Cause of anti-GBM disease?
Anti-glomerular basement membrane antibodies against type 4 collagen present in kidneys and lung alveoli Associated with HLA DR2
35
Who commonly gets anti-GBM disease?
Men 20-30s or 60-70s
36
Presentation of anti-GBm disease?
Pulmonary haemorrhage Rapidly progressive glomerulonephritis - rapid onset AKI or nephritis
37
Investigations for anti-GBM disease?
Urinalysis Bloods - U&Es, ABG/VBG, ANCA CXR Renal biopsy is gold standard
38
What will renal biopsy show in anti-GBm disease?
Focal and segmental necrotising crescents and linear IgG deposits along the basement membrane
39
Management of anti-GBM disease?
Plasmapheresis to remove circulating antibodies Steroids or cyclophosphamide
40
Prognosis of anti-GBM disease?
If untreated it is fatal If they have severe AKI or require dialysis it has a poor renal prognosis
41
Main complication of anti-GBm disease?
Pulmonary haemorrhage
42
What is cryoglobulinaemic vasculitis?
A types of small vessel vasculitis Causes palpable purpura, Arthralgias in hands and knees, polyneuropathy, renal involvement
43
What is hypocomplementemic urticarial vasculitis?
A rare form of small vessel vasculitis Causes recurrent episodes of urticaria on top of other vasculitis symptoms
44
What is polyarteritis nodosa?
Medium vessel vasculitis with necrotizing inflammation leading to aneurysm formation
45
Who is polyarteritis nodosa most common in?
Middle-aged men 40-60 Those with hep B infection
46
Presentation of polyarteritis nodosa?
Constitutional - fever, malaise, weight loss Hypertension Arthralgias Mononeuritis multiplex or sensorimotor polyneuropathy Testicular pain Livedo reticularis or palpable purpura Mesenteric arteritis causes abdominal angina Haematuria or renal failure
47
Investigations for polyarteritis nodosa?
Urinalysis - blood/protein CRP or ESR FBC will show anaemia and thrombocytopenia Hep B surface antigen may be positive Biopsy of affected tissue
48
Treatment of PAN?
Mild-moderate - oral corticosteroids Severe - high-dose pulsed IV corticosteroids Additional immunotherapy is usually required e.g. methotrexate
49
Who does Kawasaki disease typically affect?
Children
50
Presentation of Kawasaki disease?
High-grade fever lasting >5 days and resistant to anti-pyretics Conjunctival injection Bright red cracked lips Strawberry tongue Cervical lymphadenopathy Red palms of hands and soles which later peel
51
Investigtaions for Kawasaki disease?
It’s a clinical diagnosis Bloods for CRP and ESR may be done Echocardiogram is done due to risk of coronary artery aneurysm
52
Management of Kawasaki disease?
High dose aspirin IV immunoglobulin
53
Prognosis of Kawasaki disease?
Usually takes a few weeks to heal
54
Complication of Kawasaki disease?
Coronary artery aneurysm
55
What is takayasu’s arteritis?
A chronic granulomatous vascultiis affecting the large arteries - primarily the aorta and its main branches
56
Who does takayasu’s arteritis affect?
Young Females 10-40 Asian people
57
Pathophysiology of takayasu’s arteritis?
Vascular inflammation causes stenosis, occlusion and aneurysm formation
58
What is takayasu’s arteritis also known as?
Pulseless disease
59
Presentation of takayasu’s arteritis?
systemic features of a vasculitis e.g. malaise, Arthralgias, headache unequal blood pressure in the upper limbs carotid bruit and tenderness absent or weak peripheral pulses upper and lower limb claudication on exertion aortic regurgitation (around 20%)
60
Investigtaions for takayasu’s arteritis?
FBC - normocytic anaemia Raised CRP/ESR Magnetic resonance angiography or CTA are diagnostic
61
Management of takayasu’s arteritis?
Steroids
62
What is giant cell arteritis?
Temporal arteritis A vasculitis of an unknown cause that affects medium and large0sized vessels
63
Who is temporal arteritis most common in?
>60s Most commonly females Caucasians have highest rates
64
What condition is temporal arteritis associated with?
Polymyalgia rheumatica - 50% will have features of PMR
65
Presentation of temporal arteritis?
Weeks of headache, jaw claudication, tender palpable temporal artery Scalp tenderness May have amaurosis fugax or prememntn visual loss Systemic features - lethargy, depression, low-grade fever, anorexia, night sweats
66
How does temporal arteritis affect the eyes?
It causes anterior ischaemic optic neuropathy resulting from occlusion of the posterior ciliary artery which is a branch of the ophthalmic artery Causes temporary visual loss and then permenant visual loss May also cause diplopia from involvement of any part of the oculomotor system
67
Most common serious complication of temporal arteritis?
Irreversible blindness due to optic nerve ischaemia
68
Investigtaions for temporal arteritis?
CPR/ESR FBC - may have normochromic, normocytic anaemia LFTs - 1/3rd may have midlly deranged Vascular USS Temporal artery biopsy if USS not possible
69
Findings on temporal artery biopsy in temporal arteritis?
Granulomatous inflammation of the inner half of the media with infiltration of inflammatory cells, including giant cells May have skip lesions so 3-5cm of artery should be taken
70
Doppler USS findings for temporal arteritis?
Halo sign
71
Management of temporal arteritis?
Urgent high-dose glucocorticoids (before temporal artery biopsy) Urgent ophthalmology review
72
Which steroids should be used for temporal arteritis?
No visual loss = high-dose prednisolone Visual loss = IV methylprednisolone before starting high-dose prednisolone
73
What is Behçet’s syndrome?
A complex multisystem disorder with autoimmune mediated inflammation of arteries and veins
74
Epidemiology of Behçet’s syndrome?
Eastern Mediterranean Men Young adults 20-40 30% have positive FHx
75
Features of Behçet’s syndrome?
Oral ulcers Genital ulcers Anterior uveitis Also: thrombophlebitis, DVTs, arthritis, neurological involvement, GI symptoms, erythema nodosum
76
What is pathergy? What can it be seen in?
A skin condition in which a minor trauma e.g. a bump can lead to the development of skin lesions or ulcers Seen in Behçet’s disease and pyoderma gangrenosum
77
Which antigen is associated with Behcets syndrome?
HLA B51

MUSCULOSKELETAL (13 decks)

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