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RACP Immunology 2020 > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Large vessel vasculitis

A

Takayasu

Giant Cell Arteritis

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2
Q

Medium vessel vasculitis

A

Kawasaki Arteritis

Polyarteritis Nodosa

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3
Q

ANCA associated small/medium vessel vasculitis

A

Microscopic Polyangitis

Granulomatosis Polyangitis

Eosinophilic Granulomatosis Polyangitis.

Some drug induced vasculitis

Rheumatoid arthritis

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4
Q

Immune complex small vessel vasculitis

A

IgA vasculitis

antiC1q vasculitis

Anti glomerular basement membrane disease

Cryoglobulinaemic vasculitis

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5
Q

Which vasculitis produce granulomas?

A

Large: GCA, Takayasu

Small: GPA and EGPA

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6
Q

History consistent with systemic vasculitis

A

Age:

  • 45-50 for GPA and PAN
  • 17 o 26 for Takayasu or IgA vasculitis
  • > 65 for GCA
Fever
Fatigue
Weight loss
Arthralgia
Any recent drug use
Recent dx of hepatitis

Isolated symptoms:

  • Eyes: episcleritis
  • Lungs: epistaxis, pulm haemorrhage
  • motor neuropathy
  • pulmonary/renal syndrome
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7
Q

Clinical features of systemic vasculitis

A
  1. mononeuritis multiplex) +/- peripheral symmetric/asymmetric polyneuropathy.
  2. Palpable purpura (often in small vessel)
  3. Pool vascular exam (pulse, bruit, BP discrepancy)
  4. Multiorgan disease w/ systemic features
  5. Pulmonary/renal disease (haemoptysis haematuria)
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8
Q

Ddx of vasculitis

A 
thrombocytopenia
infective endocarditis
septicaemia
amyloidosis
cholestrol emboli
atrial myxoma with emboli
mycotic aneurysm w/ emboli
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9
Q

Investigations in suspected vasculitis

A

U+E, LFT, CRP/ESR
CBC
Coags (PT/APTT)
MSU

ANCA (anti MPO and PR3) -> 95% +ve for AAV

ANA ?Rheumatology disease (e.g. SLE)
Rh F
AECA
Lupus anticoagulant
Anticardiolipin antibodies
Cryoglobulin

C3 and C4

Hepatitis B, C, EBV/CMV

CXR
Arteriography (PAN, Takayasu, GCA)
USS (GCA)
CT
MRI
PET

+/- biopsy
+/- immunoflurescence for IgA

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10
Q

Other classification of vasculitis

A
  1. Variable vessel disease (behcet’s and cogan’s)
  2. Singla organ vasculitis (CNS vasculitis, leucocytoclastic vasculitis)
  3. Vasculitis with systemic disease - e.g. SLE, RA
  4. Vasculitis with probable etiology - e.g. in Hep C related cryoglobulinaemic vasculitis. Hep B assoc. polyarteritis ndosa. Hydralazine ANCA associated vasculitis.
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11
Q

Behcet’s disease

A

autoinflammation of arteries and veins of all sizes.

c/f: recurrent oral and genital ulcers, associated with venous thrombosis.

2/4 features:

  • ocular inflammation
  • genital ulcers
  • pathergy reaction
  • skin lesion (pustules, erythema nodosum
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12
Q

Diagnosis criteria of leucocytoclastic (hypersensitivity) vasculitis

A

3/5:

  • age >16
  • temporal relationship with drug
  • palpable purpura
  • maculopapular rash
  • perivascular neutrophils on skin biopsy

No treatment needed, just stop drug.

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13
Q

Common drugs causing hypersensitivity vasculitis

A 
sulfonamide (frusemide, thiazide)
penicillin
cephalosporin
allopurniol
phenytoin
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14
Q

Takayasu Vasculitis

A

Inflammation of aorta

ARA criteria:
3/6 of the following:
- <40
- claudication of extremities (>1 pulsation of brachial pulse).
- >10mmHg SBP b/w arms
- Bruit over subclavian/abdo aorta or both

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15
Q

Giant cell arteritis

A

> 50 yo
Absence of exclusion criteria (eye, kidney, skin, nerve, lung, LN)

3/11 points:

  • new localised headache
  • sudden onset of visual disturbance
  • PMR
  • jaw claudication
  • abnormal temporal artery
  • unexplained fever, anaemia
  • ESR >50mm/hr
  • compatible pathology
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16
Q

Halo sign on USS

A

Circumferential wall thickening due to vasculitis wall edema

Indicates GCA

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17
Q

Imaging for GCA

A 
USS
CT scan
MRI
PET
But always need biopsy
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18
Q

How can biopsies be negative if someone has clinical features of GCA?

A

Not long enough

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19
Q

Polyarteritis Nodosa

what is it associated with?

A

Affects medium sized visceral arteries at bifurcations/aneurysms leading to organ ischaemia, peripheral neuropathy but no systemic features.

Associated with hepatitis B

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20
Q

PAN is associated with which genetic mutation?

A

AR mutation in ADA2 gene.

(vs. SCID due to ADA1 deficiency).

Milder phenotype - often skin and CNS, kidney w/ RTA and aneurysms.

Tx: antiTNFa, HSCT

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21
Q

Kawasaki disease

A

Mainly in infants

22
Q

What is ANCA?

A

Anti neutrophil cytopasmic antibody

23
Q

Why do you test for ANCA?

A

To diagnose small vessel vasculitis initial presentation (not remission when the epitope may be different)

  • exclude small vessel vasculitis
  • monitor activity in small vessel vasculitis
  • help dx IBD, drug induced vasculitis, rheumatoid vasculitis.
24
Q

What is C-ANCA?

A

Cytoplasmic granular fluorescence with central accentuation.

directed against proteinase 3 (PR3).

90% positive in GPA
30% positive in MPA

25
Q

What is p-ANCA?

A

Perinuclear with nuclear extension.

Directed against myeloperoxidase (MPO)

70% active in MPA
10% active in GPA

Other P-ANCA in IBD is directed against elastase, cathepsin G, lactoferrin, HMG protein.

26
Q

Clinical significance of ANCA in WG and MPA

A

high level at presentation
level fall with treatment
high levels prior to relapse
many patients with recurrent ANCA relapse and have sublclinical disease

27
Q

Pathogenesis of ANCA related disease.

A

Infection causes neutrophils to express ANCA antigens (MPO or PR3).

ANCA binds to its antigen causing PMN to degranulate and release ROS and NETS.

NETS cause:

  • damage of endothelial cells from release of PR3/MPO
  • activate complement
  • thrombosis through expression of histone and tissue factor.
  • link innate and adaptive immune system.
28
Q

Which pathway of the complement system is activate via NETS

A

Alternative pathway - generating C5a.

C5a attract more neutrophils and primes neutrophils to display ANCA antigens.

29
Q

Other autoantibodies positive in GPA and MPA

A

ANA 15%
Lupus anticoagulant 10%
Anticardiolipin 10%
AntiGBM <5%

30
Q

What is Granulomatosis with polyangitis?

A

Granulomatous vasculitis of upper and lower respiratory tract w/ glomerulonephritis +/- variable degree of disseminated vasculitis of small arteries and veins

31
Q

Which ANCA is associated with GPA?

A

c-ANCA (anti-PR3 ANCA) 90%

pANCA (small proportion)

20% no ANCA

32
Q

Clinical features of Limited GPA

A

affects eyes, ears, nose, sinuses, upper/lower airway, large joint arthralgia

70% + ANCA, 90% develop renal disease.

33
Q

Clinical features of generalised GPA

A

rapidly progressing pauci-immune glomerulonephritis

+/- multiorgan involvement.

34
Q

Describe correlation between abnormal alpha 1 antitrypsin phenotype and GPA

A

α1AT inhibits PR3; abnormal phenotypes
result in uninhibited PR3 which is more
immunogenic

35
Q

How do you diagnose GPA?

A
  1. TIssue biopsy:
    Lung - necrotising granulomatous vasculitis
  2. Positive antiPR3-ANCA.
  3. on CT chest: multiple bilateral and cavity infiltrate
36
Q

GPA treatment

A

PO cyclophosphamide 3/12
then PO azathioprine (superior mycophenolate)
Prednisolone

37
Q

What treatment can be used for those with poorly controlled GPA?

A

rituximab together with cyclophosphamide (RITUXIVAS and RAVE study)

however, increased the risk of relapse after 18 months.

38
Q

Other targets for induction therapy of GPA

A

B cells:

  • rituximab
  • belimumab (anti B cell activating factor)

Complement activation:
- antiC5a AB and antiC5aR antibodies

Cytokines:

  • anti-TNF
  • anti-IL6R

Neutrophil activation:
cathepsin G

39
Q

Microscopic Polyangitiis

A

necrotising vasculitis with few or no immune complex affecting small vessels. No granulomatous formation.

> 57yo onset, M>F

40
Q

Which ANCA is associated with MPA?

A

pANCA (75%)

Can be negative ANCA but no change in C/F.

41
Q

Clinical features of MPA

A 
Fever, weight loss, MSK pain
Glomerulonephritis (pauciimmune)
Pulmonary/alveolar haemorrhage
MOnoneuritis multiplex 
GI+cutaneous vasculitis
Raised ESR, anaemia, leukocytosis, thrombocytosis.
42
Q

WHat does biopsy of MPA show?

A

vasculitis

pauciimmune GN

43
Q

What disease is Polyarteritis nodosa associated with?

A

Hep B
Hep C
Hairy Cell Leukaemia

44
Q

Clinical features of Polyarteritis Nodosa

A 
Systemic (fever, weight loss)
ANCA negative
Raised IgG (hypergammaglobulinaemia)
Maybe  HepB/Hep C positive
Poor prognosis if not treated (from bowel infarct, MI)
45
Q

Treatment of PAN

A

Steroids + cyclophosphamide.

OFten can improve with steroids only

46
Q

Clinical features of MPA/PAN

A

50% of patients with MPA

Pauci imune glomerulonephritis
Bowel infarction
Peripheral neuropathy
ANCA +

47
Q

Clinifal features of eosinophilic granulomatosis polyangitis

A 
Hall mark:
asthma
eosinophilia
extravascular granuloma
systemic vasculitis
(fever, mononeuritis multiplex, glomerulonephritis, raised inflammatory markers)
48
Q

Maintenance treatment of ANCA associated vasculitis

A

3 yrs in GPA
2 yrs in MPA

Azathioprine or MTX
Cyclosporine+MMF inferior, more relapse
Rituximab - more relapse + cancer

49
Q

How do you minimise side effect of medications?

A

Immunosuppression: shortest course possible.

Infection: cotrim 2x a week,
steroids: alt day dosing, PPI, colecalciferol, fosamax

50
Q

Decrease risk of relapse by

A

(uncommon on HD cyclophosphamide/other immunosuppresant.)

using cyclophosphamide during induction
6/12 post presentation or relapse
use azathioprine in tx

51
Q

Cause of drug induced vasculitis

A 
propylthiouracyl
hydralazine
allopurinol
sulphasalazine
Minocycline
Cefotaxime
Ciprofloxacin
Clonazepam
52
Q

What other diseases can ANCA be associated with?

A

IBD
Arthritis
LUng disease (90% in CF)

RACP Immunology 2020 (5 decks)

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