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Immunology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

GCA treatment
Visual threatening
Visual non-threatening

A

Visual threatening - IV methylprednisolone 500-1000mg 3/7

Visual non-threatening - PO prednisolone 50mg

Start ASAP while working up patient

Adjunct

  • PPI for gastric protection
  • Calcium, vitamin D
  • Low dose aspirin for increased CV and CVA events
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2
Q

GCA affects what sized arteries?

A

Medium to large vessel vasculitis of the elderly

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3
Q

5 cardinal symptoms GCA

A

Headache
Jaw claudication (after 1 min of chewing)
Vision disturbance (mononuclear)
Constitutional upset - weight loss, night sweats, fever
PMR - hip, shoulder girdle inflammatory pain

96% have elevated CRP or ESR

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4
Q

GCA affects which vessels?

A

Superficial cranial arteries (vision loss)
Aorta
Aortic branches, subclavian, axillary - UL claudication symptoms

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5
Q

Diagnosis of GCA

A

Temporal artery biopsy (gold standard)

  • Up to 2 weeks post steroid
  • False negative in 20% of patients (skip lesions or not affect the temporal artery at all)
  • Do both sides

Others
PET/CT angiogram
- Within 2-4 days of starting steroids
- Useful for large vessel vasculitis

Vascular USS

  • Within 2-4 days of starting steroids
  • Superficial temporal artery and axillary arteries
  • Halo sign (wall thickening)

High resolution MRI with GAD

If PET/CT scan and vascular USS are floridly positive for GCA, then technically don’t need temporal artery biopsy (but in reality we do, for PBS approval)

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6
Q

Tocilizumab (IL6 blocker) in GCA

What’s the evidence? When is it used?

A

Historically, we do 12-24 months prednisolone wean. ~50% relapse rate.

Tocilizumab reduces rate of relapse when added to prednisolone (20% vs 50%)
Used in conjunction with pred to reduce duration of pred (only need to give 6/12 of pred)
*Particularly useful in OP, diabetes, steroid SEs (where we want to avoid long pred use)

PBS approves only 12 months
Note when we stop tocilizumab, we do see flare in GCA

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7
Q

3 vascular complications of GCA

A

1) permanent vision loss in >25% untreated patients
- Anterior ischaemic optic neuropathy

2) aortic aneurysm and dissection

3) peripheral and CVA
- Stroke
- Limb claudication

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8
Q

Presentation of Takayasu arteritis

A 
Limb claudication 
Reduced/absent pulse in the upper extremity  
Differential limb SBP >10mmHg
Aortic/subclavian artery bruit 
All from arteritis --> vessel narrowing

Fever, night sweats, weight loss

Asian female <40
Elevated inflammatory markers, negative autoimmune serology

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9
Q

Rx takayasu arteritis

A

Steroids +/- TNFi, MTX, lefluonamide, tocilizumab (IL6 blocker)

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10
Q

Polyarteritis nodosa presentation

A

Constitutional symptoms
Affects MANY organs (organ ischaemia/infarction) except lungs
- Hypertension, renal failure (renal artery)
- Abdo pain with melena (mesenteric artery)
- Neurological sx
- Peripheral neuropathy (nerves supplied by medium vessels)
- Painful, ulcerating rash
- Limb claudication
- ACS
- Orchitis

Very rare

Elevated inflammatory markers
NEGATIVE autoimmune serology (neg ANCA)
Associated with hep B, hep C

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11
Q

Diagnosis polyarteritis nodosa

A

Biopsy: skin, nerve/muscle, kidney (necrosis)

CT or MR angiogram: distal mesenteric and intrarenal aneurysm

Angiogram is gold standard

Has a string of pearls appearance due to healing with fibrosis (Dont confuse with fibromuscular dysplasia!)

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12
Q

6 groups of small vessel vasculitis

A

1) ANCA: GPA, MPA, EGPA
2) CTD related: RA, SLE, Sjorens
3) Other immune complex: HSP (IgA), cryoglobulin
4) Infection: Hep B, C, HIV, bacterial (e.g. strep/staph)

5) Drug induced
- Cocaine
- PTU, hydralazine, allopurinol, sulphasalazine, frusemide, minocycline, ciprofloxacin, clozapine
- P-ANCA +, high MPO

6) Malignancy: haematological most common

And some are idiopathic

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13
Q

Clinical features of small vessel vasculitis

A

Constitutional upset: fever, weight loss, sweats

Skin: palpable purpura

Joints: non erosive inflammatory arthritis

Nerves: painful multifocal peripheral neuropathy (foot drop, wrist drop)

ENT: bloody nasal DC, daily crusting, bony destruction on CT (cocaine, GPA, invasive fungal sinus disease)

Kidneys: active urine sediment, hypertension, creatinine

Lungs: alveolar haemorrhage, lung masses

Eyes: scleritis, orbital masses

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14
Q

Palpable purpura (leukocytoclastic vasculitis)
Pathophysiology
Biopsy findings

A

Capillary wall inflammation –> extravasation of blood products and inflammatory cell debris

3 features on biopsy

  • Fibrinoid necrosis (fibrin in wall)
  • Neutrophil dust - leukocytoclastic
  • Extravasated RBCs

If positive IgA staining = IgA vasculitis (HSP)

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15
Q

Clinical features of palpable purpura

A

Dependent
Purpuric, non-blanching (red cell extravasation)
Palpable/elevated (inflammatory cells in skin)
Painless (unless deeper vessels involved)
- Think deeper vessels (polyarteritis nodosa; medium sized vasculitis) if painful/ulcerative

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16
Q

Small vessel vasculitis workup

A

ANCA
- ANCA (MPO, PR3), GBM (if renal, lung)

CT disease panel
- ANA, ENA, dsDNA, C3, C4, RF, CCP

Infection
- Hep B, C, HIV, strep serology, BCs

Cryoglobulinaemia
- Cryoglobulin

Malignancy
- FBC +/- SPEP

Everyone

  • Urine microscopy and ACR
  • FBC, CRP, ESR, UEC, LFTs
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17
Q

ANCA associated vasculitis (3 types)

A

All small vessel vasculitis

1) Granulomatosis with polyangiitis (GPA)
- c-ANCA, PR3+

2) Microscopic polyangiitis (MPA)
- p-ANCA, MPO+

3) Eosinophilic granulomatosis with polyangiitis (EGPA)
- p-ANCA, MPO+

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18
Q

Treatment GPA and MPA with major organ involvement (kidney, lung, nerve)

A

Induction (6 months)

  • Rituximab
  • Low dose steroid
  • No role for routine plasmapheresis

Maintenance (4 years+)
- Rituximab

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19
Q

Treatment GPA and MPA with minor organ involvement e.g. palpable purpura, some sinus disease

A

Induction: MTX + steroid

Maintenance: MTX or azathioprine

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20
Q

Treatment of EGPA major organ involvement (cardiac, renal, CNS, GIT)

A

Induction

  • Rituximab
  • Steroid

Maintenance
- Azathioprine or MTX

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21
Q

Treatment of EGPA minor organ involvement (lung, sinus, skin)

A

Induction
Steroid +/- Mepolizumab (IL5 inhibitor)

Maintenance
Mepolizumab or azathioprine

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22
Q

IgA vasculitis (HSP) clinical presentation

A

Clinical tetrad

  • Palpable purpura
  • Arthritis
  • Nephritis
  • Abdo pain (GIT vasculitis)

NEED positive IgA staining on skin or renal biopsy

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23
Q

Treatment IgA vasculitis (HSP)

A

Self limiting
Supportive care only

No steroid or immunosuppressant (unless highly aggressive)

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24
Q

Cryoglobulins - what are they?

A

ab that precipitate in the cold

Dissolves with rewarming

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25
Q

Cryoglobulinemic vasculitis is associated with …

A

HCV

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26
Q

Treatment Cryoglobulinemic vasculitis

A

If associated with HCV –> HCV therapy +/- immunosuppression

Idiopathic –> immunosuppression (steroids +/- rituximab, plasma exchange)

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27
Q

How to differentiate between polyarteritis nodosa with fibromuscular dysplasia?

A

Fibromuscular dysplasia

  • Non-inflammatory vasculopathy
  • Affects renal and carotid arteries
  • More proximal aneurysms rather than distal
  • Imaging: renal and carotid artery beading (no intrarenal aneurysms)
  • Neg inflammatory markers
  • HTN, pulsatile tinnitus
  • No systemic features of vasculitis
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28
Q

3 CTD vasculitis

A

RA
SLE
Sjogren’s

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29
Q

Treatment for CTD associated vasculitis with major organ involvement

1) SLE
2) RA
3) Sjogrens

A

SLE - similar to lupus nephritis (mycophenolate or cyclophosphamide)

RA - rituximab

Sjogren’s - mycophenolate or rituximab

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30
Q

Management of infection or drug induced vasculitis

A

Treat infection or withdraw causative drug

Supportive care

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31
Q

How does cryoglobulinemic vasculitis present?

A

Skin purpura with necrosis
Leukocytoclastic vasculitis (most common)
Raynaud’s
Peripheral neuropathy
MPGN
Can cause vascular obstruction in the extremities –> lose fingers and toes

Occurs with the cold

Low C4 (complement is fixed in this condition)

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32
Q

Diagnosis of takayasu arteritis

A

CT angiogram or MR angiogram
- Look for stenosis, beading

PET scan is very sensitive but $$$ so not readily available

Angiogram is gold standard but not done commonly

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33
Q

What sized arteries does Takayasu arteritis affect?

A

Large vessel granulomatous vasculitis

Aorta and primary branches

34
Q

Polyarteritis nodosa which sized vessel does it affect?

A

Medium vessel vasculitis –> inflammation, luminal narrowing and stenosis, aneurysms, thrombosis, necrosis

35
Q

Treatment polyarteritis nodosa

A

Pred + cyclophosphamide –> azathioprine maintenance

Refractory: infliximab

36
Q

Granulomatosis with polyangiitis (GPA) presentation

A

Constitutional symptoms
ENT - sinusitis, epistaxis
Pulmonary - recurrent haemoptysis
Renal - rapid progressive pauci-immune crescenteric GN

37
Q

Granulomatosis with polyangiitis (GPA) diagnosis

A

Lab: ANCA+, PR3+ (can be used to monitor disease)

Biopsy is gold standard: necrotising granulomatous inflammation

38
Q

Granulomatosis with polyangiitis (GPA) treatment

A

Limited: oral pred + aza/MTX

Severe systemic: pred + IV cyclo (better tolerated than PO)

Rituximab in difficult to control disease

39
Q

Eosinophilic granulomatosis with polyangiitis (EGPA) presentation

A

Common background of asthma or allergic rhinitis (eosinophil component)

Mononeuritis multiplex - foot drop, hand drop (ANCA +)
Kidneys
Cardiomyopathy (ANCA -)

40
Q

Eosinophilic granulomatosis with polyangiitis (EGPA) diagnosis

A

P-ANCA + (only in 50%), MPO +

41
Q

Eosinophilic granulomatosis with polyangiitis (EGPA) treatment

A

Pred + cyclo

Mepolizumab (IL5 blocker) in refractory cases

42
Q

Anti-GBM disease diagnosis

A

Anti-GBM high
Renal biopsy (urgent)
Often pANCA +, MPO+

43
Q

Anti-GBM disease presentation

A

Pulmonary

Renal - rapidly progressive crescenteric GN

44
Q

Treatment anti-GBM disease

A

Pred + Cyclo + plasma exchange

45
Q

HSP (IgA vasculitis) presentation

A

Often in childhood
In the setting of infection (beta haemolytic strep)

Abdo pain
Nephritis (most severe complication)
Palpable purpura
Arthritis

46
Q

Treatment HSP (IgA vasculitis)

A

Generally self limited

Steroids can help if abdo pain, arthralgia
No role of steroids in preventing nephritis

Aza, Cyclo, rituximab for severe manifestation

47
Q

Side effects of MTX

A

Hepatotoxicity, myelosuppression, pulmonary fibrosis

48
Q

Side effects of azathioprine

A

Hepatotoxicity, myelosuppression (check TPMT), skin cancers

49
Q

Side effects of MMF

A

Very well tolerated

GIT (diarrhoea), hepatotoxicity

50
Q

Side effects of cyclophosphamide

A

LOTS
We tend to avoid in induction therapy in vasculitis as rituxumab is just as effective (due to side effects)

Amenorrhoea, infertility, teratogenicity, bladder toxicity, infection, myelosupression, cancers

Avoid in women of childbearing ae

51
Q

Side effects of rituximab

A

Well tolerated

Infusion reactions, reactivation of latent infections, PML (usually in concomitant chemotherapy in cancer)

52
Q

Name 2 large vessel vasculitis

A

GCA

Takayasu arteritis

53
Q

Name 3 medium vessel vasculitis

A

Polyarteritis nodosa
Kawasaki disease
Buerger disease

54
Q

Which part of the body does Buerger disease involve?

A

Fingers and toes!

Ulceration, gangrene and autoamputation

55
Q

Buerger disease is associated with …

A

Smoking

Treatment is smoking cessation!!

56
Q

Middle aged man with sinusitis, nasopharyngeal ulceration, bilateral pulmonary infiltrates with haemoptysis, haematuria. What’s the dx?

A 
Granulomatous polyangiitis (GPA)
Haematuria is due to rapidly progressive GN
57
Q

Middle aged man with leucocytoclastic vasculitic rash, haemoptysis and haematuria. What’s the diagnosis?

A

Microscopic polyangiitis (MPA)

leucocytoclastic vasculitic rash AND systemic involvement = MPA

Presents similarly to GPA but no nasopharyngeal involvement

58
Q

Henoch-Schonlein purpura is due to…

A

IgA immune complex deposition

59
Q

Henoch-Schonlein purpura presentation

A

Palpable purpura on legs and buttocks
Abdo pain + GI bleeding
Haematuria (IgA nephropathy)

Usually after URTI

60
Q

PR3 ANCA are associated with …

A

More frequent relapses

ENT, upper respiratory tract disease

61
Q

If someone is on cyclophosphamide and develops haematuria. What do you worry about?

A

Bladder cancer

62
Q

Leucocytoclastic vasculitis is dependent lower limb palpable purpura. It is commonly caused by…

A

Frusemide
Abx
Allopurinol
Phenytoin

63
Q

What must you do if you see a leucocytoclastic vasculitic rash?

A

Test urine for haematuria to exclude systemic involvement - i.e. microscopic polyangiitis

64
Q

Treatment of leucocytoclastic vasculitis

A

Usually enough just to stop the drug

65
Q

How does microscopic polyangiitis present?

A 
Palpable purpura (leucocytoclastic vasculitic rash) + systemic involvement 
- GN, pulmonary haemorrhage, neuropathy
66
Q

Behcet’s disease occur in people of what nationality?

A

Turkish

67
Q

Behcet’s disease is vasculitis of what sized vessels?

A

All sizes

Both arteries and veins

68
Q

Behcet’s disease presentation

A

Recurrent oral ulcers*** (must have)

Ocular inflammation
Genital ulcers
Pathergy reaction (exaggeration skin reaction after minor trauma)
Skin lesions - pustules, erythema nodosum
Venous thrombosis

69
Q

Behcet’s disease treatment

A

Colchicine

Steroids

70
Q

How does kawasaki disease present?

A 
Asian infant
Fever 
Bilateral conjunctivitis
Erythema/desquamation of palms and soles
Enlarged cervical lymph nodes
Coronary artery aneurysm with rupture; thrombosis and MI 
Rash
71
Q

Treatment of kawasaki disease

A

IVIG

aspirin

72
Q

MPO ANCA is associated with what kind of presentation?

A

Haematuria and GN

73
Q

What’s the difference between limited and generalised GPA?

A

Limited: affects ENT, lungs, large joints

Generalised: also RPGN

90% limited will go on to develop generalised

74
Q

What’s the relationship between GPA and Alpha-1-antitripsin?

A

Alpha-1-antitripsin mops up PR3 and other proteolytic enzymes
Less alpha-1-antitripsyin means more damage
ANCA prevents alpha-1-antitripsin from mopping up PR3

75
Q

General treatment of ANCA vasculitis

A

Induction with Pred and cyclophosphamide
Maintenance with pred and azathioprine

Rituximab in poorly controlled disease

76
Q

Polyarteritis nodosa can overlap with which other type of vasculitis?

A

MPA

50% of MPA have PAN
All have GN
Often have bowel infarction or peripheral neuropathy
P-ANCA positive

77
Q

Presentation of drug-induced ANCA associated vasculitis

A

Mild disease - stop the drug, steroids for symptomatic relief if necessary

Multiorgan disease (crescenteric GN) - short course steroids and possibly immunosuppressants

78
Q

How soon do you expect people to improve after starting treatment for vasculitis?

A

Within days

79
Q

Most common location of occlusion in vision loss in GCA

A

Posterior ciliary artery - 80%

Causing anterior ischaemic optic neuropathy

80
Q

What do you expect to find on temporal artery biopsy in GCA?

A

Mononuclear cell infiltration
Granulocyte inflammation
Multinucleated giant cell

Immunology (6 decks)

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