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RACP Immunology 2020 > Vasculitis > Flashcards

Vasculitis Flashcards

(52 cards)

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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

Study These Flashcards
A

Mainly in infants

22
Q

What is ANCA?

Study These Flashcards
A

Anti neutrophil cytopasmic antibody

23
Q

Why do you test for ANCA?

Study These Flashcards
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?

Study These Flashcards
A

Cytoplasmic granular fluorescence with central accentuation.

directed against proteinase 3 (PR3).

90% positive in GPA
30% positive in MPA

25
What is p-ANCA?
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
Clinical significance of ANCA in WG and MPA
high level at presentation level fall with treatment high levels prior to relapse many patients with recurrent ANCA relapse and have sublclinical disease
27
Pathogenesis of ANCA related disease.
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
Which pathway of the complement system is activate via NETS
Alternative pathway - generating C5a. C5a attract more neutrophils and primes neutrophils to display ANCA antigens.
29
Other autoantibodies positive in GPA and MPA
ANA 15% Lupus anticoagulant 10% Anticardiolipin 10% AntiGBM <5%
30
What is Granulomatosis with polyangitis?
Granulomatous vasculitis of upper and lower respiratory tract w/ glomerulonephritis +/- variable degree of disseminated vasculitis of small arteries and veins
31
Which ANCA is associated with GPA?
c-ANCA (anti-PR3 ANCA) 90% pANCA (small proportion) 20% no ANCA
32
Clinical features of Limited GPA
affects eyes, ears, nose, sinuses, upper/lower airway, large joint arthralgia 70% + ANCA, 90% develop renal disease.
33
Clinical features of generalised GPA
rapidly progressing pauci-immune glomerulonephritis | +/- multiorgan involvement.
34
Describe correlation between abnormal alpha 1 antitrypsin phenotype and GPA
α1AT inhibits PR3; abnormal phenotypes result in uninhibited PR3 which is more immunogenic
35
How do you diagnose GPA?
1. TIssue biopsy: Lung - necrotising granulomatous vasculitis 2. Positive antiPR3-ANCA. 3. on CT chest: multiple bilateral and cavity infiltrate
36
GPA treatment
PO cyclophosphamide 3/12 then PO azathioprine (superior mycophenolate) Prednisolone
37
What treatment can be used for those with poorly controlled GPA?
rituximab together with cyclophosphamide (RITUXIVAS and RAVE study) however, increased the risk of relapse after 18 months.
38
Other targets for induction therapy of GPA
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
Microscopic Polyangitiis
necrotising vasculitis with few or no immune complex affecting small vessels. No granulomatous formation. >57yo onset, M>F
40
Which ANCA is associated with MPA?
pANCA (75%) | Can be negative ANCA but no change in C/F.
41
Clinical features of MPA
``` Fever, weight loss, MSK pain Glomerulonephritis (pauciimmune) Pulmonary/alveolar haemorrhage MOnoneuritis multiplex GI+cutaneous vasculitis Raised ESR, anaemia, leukocytosis, thrombocytosis. ```
42
WHat does biopsy of MPA show?
vasculitis | pauciimmune GN
43
What disease is Polyarteritis nodosa associated with?
Hep B Hep C Hairy Cell Leukaemia
44
Clinical features of Polyarteritis Nodosa
``` Systemic (fever, weight loss) ANCA negative Raised IgG (hypergammaglobulinaemia) Maybe HepB/Hep C positive Poor prognosis if not treated (from bowel infarct, MI) ```
45
Treatment of PAN
Steroids + cyclophosphamide. | OFten can improve with steroids only
46
Clinical features of MPA/PAN
50% of patients with MPA Pauci imune glomerulonephritis Bowel infarction Peripheral neuropathy ANCA +
47
Clinifal features of eosinophilic granulomatosis polyangitis
``` Hall mark: asthma eosinophilia extravascular granuloma systemic vasculitis (fever, mononeuritis multiplex, glomerulonephritis, raised inflammatory markers) ```
48
Maintenance treatment of ANCA associated vasculitis
3 yrs in GPA 2 yrs in MPA Azathioprine or MTX Cyclosporine+MMF inferior, more relapse Rituximab - more relapse + cancer
49
How do you minimise side effect of medications?
Immunosuppression: shortest course possible. Infection: cotrim 2x a week, steroids: alt day dosing, PPI, colecalciferol, fosamax
50
Decrease risk of relapse by
(uncommon on HD cyclophosphamide/other immunosuppresant.) using cyclophosphamide during induction 6/12 post presentation or relapse use azathioprine in tx
51
Cause of drug induced vasculitis
``` propylthiouracyl hydralazine allopurinol sulphasalazine Minocycline Cefotaxime Ciprofloxacin Clonazepam ```
52
What other diseases can ANCA be associated with?
IBD Arthritis LUng disease (90% in CF)

RACP Immunology 2020 (5 decks)

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