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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
Cryoglobulinemic vasculitis is associated with ...
HCV
26
Treatment Cryoglobulinemic vasculitis
If associated with HCV --> HCV therapy +/- immunosuppression | Idiopathic --> immunosuppression (steroids +/- rituximab, plasma exchange)
27
How to differentiate between polyarteritis nodosa with fibromuscular dysplasia?
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
28
3 CTD vasculitis
RA SLE Sjogren's
29
Treatment for CTD associated vasculitis with major organ involvement 1) SLE 2) RA 3) Sjogrens
SLE - similar to lupus nephritis (mycophenolate or cyclophosphamide) RA - rituximab Sjogren's - mycophenolate or rituximab
30
Management of infection or drug induced vasculitis
Treat infection or withdraw causative drug | Supportive care
31
How does cryoglobulinemic vasculitis present?
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)
32
Diagnosis of takayasu arteritis
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
33
What sized arteries does Takayasu arteritis affect?
Large vessel granulomatous vasculitis | Aorta and primary branches
34
Polyarteritis nodosa which sized vessel does it affect?
Medium vessel vasculitis --> inflammation, luminal narrowing and stenosis, aneurysms, thrombosis, necrosis
35
Treatment polyarteritis nodosa
Pred + cyclophosphamide --> azathioprine maintenance Refractory: infliximab
36
Granulomatosis with polyangiitis (GPA) presentation
Constitutional symptoms ENT - sinusitis, epistaxis Pulmonary - recurrent haemoptysis Renal - rapid progressive pauci-immune crescenteric GN
37
Granulomatosis with polyangiitis (GPA) diagnosis
Lab: ANCA+, PR3+ (can be used to monitor disease) Biopsy is gold standard: necrotising granulomatous inflammation
38
Granulomatosis with polyangiitis (GPA) treatment
Limited: oral pred + aza/MTX Severe systemic: pred + IV cyclo (better tolerated than PO) Rituximab in difficult to control disease
39
Eosinophilic granulomatosis with polyangiitis (EGPA) presentation
Common background of asthma or allergic rhinitis (eosinophil component) Mononeuritis multiplex - foot drop, hand drop (ANCA +) Kidneys Cardiomyopathy (ANCA -)
40
Eosinophilic granulomatosis with polyangiitis (EGPA) diagnosis
P-ANCA + (only in 50%), MPO +
41
Eosinophilic granulomatosis with polyangiitis (EGPA) treatment
Pred + cyclo Mepolizumab (IL5 blocker) in refractory cases
42
Anti-GBM disease diagnosis
Anti-GBM high Renal biopsy (urgent) Often pANCA +, MPO+
43
Anti-GBM disease presentation
Pulmonary | Renal - rapidly progressive crescenteric GN
44
Treatment anti-GBM disease
Pred + Cyclo + plasma exchange
45
HSP (IgA vasculitis) presentation
Often in childhood In the setting of infection (beta haemolytic strep) Abdo pain Nephritis (most severe complication) Palpable purpura Arthritis
46
Treatment HSP (IgA vasculitis)
Generally self limited Steroids can help if abdo pain, arthralgia No role of steroids in preventing nephritis Aza, Cyclo, rituximab for severe manifestation
47
Side effects of MTX
Hepatotoxicity, myelosuppression, pulmonary fibrosis
48
Side effects of azathioprine
Hepatotoxicity, myelosuppression (check TPMT), skin cancers
49
Side effects of MMF
Very well tolerated GIT (diarrhoea), hepatotoxicity
50
Side effects of cyclophosphamide
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
Side effects of rituximab
Well tolerated Infusion reactions, reactivation of latent infections, PML (usually in concomitant chemotherapy in cancer)
52
Name 2 large vessel vasculitis
GCA | Takayasu arteritis
53
Name 3 medium vessel vasculitis
Polyarteritis nodosa Kawasaki disease Buerger disease
54
Which part of the body does Buerger disease involve?
Fingers and toes! | Ulceration, gangrene and autoamputation
55
Buerger disease is associated with ...
Smoking | Treatment is smoking cessation!!
56
Middle aged man with sinusitis, nasopharyngeal ulceration, bilateral pulmonary infiltrates with haemoptysis, haematuria. What's the dx?
``` Granulomatous polyangiitis (GPA) Haematuria is due to rapidly progressive GN ```
57
Middle aged man with leucocytoclastic vasculitic rash, haemoptysis and haematuria. What's the diagnosis?
Microscopic polyangiitis (MPA) leucocytoclastic vasculitic rash AND systemic involvement = MPA Presents similarly to GPA but no nasopharyngeal involvement
58
Henoch-Schonlein purpura is due to...
IgA immune complex deposition
59
Henoch-Schonlein purpura presentation
Palpable purpura on legs and buttocks Abdo pain + GI bleeding Haematuria (IgA nephropathy) Usually after URTI
60
PR3 ANCA are associated with ...
More frequent relapses | ENT, upper respiratory tract disease
61
If someone is on cyclophosphamide and develops haematuria. What do you worry about?
Bladder cancer
62
Leucocytoclastic vasculitis is dependent lower limb palpable purpura. It is commonly caused by...
Frusemide Abx Allopurinol Phenytoin
63
What must you do if you see a leucocytoclastic vasculitic rash?
Test urine for haematuria to exclude systemic involvement - i.e. microscopic polyangiitis
64
Treatment of leucocytoclastic vasculitis
Usually enough just to stop the drug
65
How does microscopic polyangiitis present?
``` Palpable purpura (leucocytoclastic vasculitic rash) + systemic involvement - GN, pulmonary haemorrhage, neuropathy ```
66
Behcet's disease occur in people of what nationality?
Turkish
67
Behcet's disease is vasculitis of what sized vessels?
All sizes | Both arteries and veins
68
Behcet's disease presentation
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
Behcet's disease treatment
Colchicine | Steroids
70
How does kawasaki disease present?
``` 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
Treatment of kawasaki disease
IVIG | aspirin
72
MPO ANCA is associated with what kind of presentation?
Haematuria and GN
73
What's the difference between limited and generalised GPA?
Limited: affects ENT, lungs, large joints Generalised: also RPGN 90% limited will go on to develop generalised
74
What's the relationship between GPA and Alpha-1-antitripsin?
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
General treatment of ANCA vasculitis
Induction with Pred and cyclophosphamide Maintenance with pred and azathioprine Rituximab in poorly controlled disease
76
Polyarteritis nodosa can overlap with which other type of vasculitis?
MPA 50% of MPA have PAN All have GN Often have bowel infarction or peripheral neuropathy P-ANCA positive
77
Presentation of drug-induced ANCA associated vasculitis
Mild disease - stop the drug, steroids for symptomatic relief if necessary Multiorgan disease (crescenteric GN) - short course steroids and possibly immunosuppressants
78
How soon do you expect people to improve after starting treatment for vasculitis?
Within days
79
Most common location of occlusion in vision loss in GCA
Posterior ciliary artery - 80% | Causing anterior ischaemic optic neuropathy
80
What do you expect to find on temporal artery biopsy in GCA?
Mononuclear cell infiltration Granulocyte inflammation Multinucleated giant cell

Immunology (6 decks)

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