Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Cardiovascular > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What are vasculitides?

A

Inflammation and subsequent necrosis of blood vessels leading to tissue ischaemia or infarction; any organ system may be involved.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

When should vasculitis be suspected?

A

Cases of unexplained multiple organ ischaemia or systemic illness with no evidence of malignancy or infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is cANCA?

A

Circulating anti-neutrophil cystoplasmic antibody associated with anti PR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is pANCA?

A

Perinuclear anti-neutrophil cytoplasmic antibody associated with multiple antigens (e.g. lactoferrin in IBD; myeloperoxidase in microscopic polyangitis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the features of small vessel vasculitis?

A
  • Palpable purpura
  • Vesicles
  • Chronic urticaria
  • Superficial ulcers
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the Churg Strauss Triad?

A
  • Allergic rhinitis and asthma
  • Eosinophilic infiltrative disease resembling pneumonia
  • Systemic vasculitis often mono neuritis multiplex / peripheral neuropathy and peripheral eosinophilia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the features of medium vessel vasculitis?

A
  • Livedo reticularis
  • Erythema nudism
  • Raynaud’s phenomenon
  • Nodules
  • Digital infarcts
  • Ulcers
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the medium vessel vasculitides?

A
  • Polyarteritis nodosa

- Kawasaki’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the large vessel vasculitides?

A
  • Giant cell /temporal arteritis

- Takayasu’s arteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How is small vessel vasculitis classified?

A

ANCA-associated and non-ANCA associated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the non-ANCA associated small vessel vasculitides?

A
  • Predominately cutenaous vasculitis
  • Henoch-Schoenlein purpura
  • Essential cryoglobulinemic vasculitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the ANCA associated small vessel vasculitides?

A
  • Granulomatosis with polyangitis (GPA; aka Wegener’s)
  • Eosniophilic granulomatosis with polyangitis (Churg Strauss Syndrome)
  • Microangiopathic polyangitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is Henoch Schonlein Purport?

A

Vascular deposition of IgA causing systemic vasculitis (skin, GI, renal); usually self limiting; most common in childhood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is essential cryoglobulinemic vasculitis?

A

Systemic vasculitis caused by circulating cryoproteins forming immune complexes. May be associated with underlying disease (e.g. Hep C) or connective tissue disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is granulomatosis with polyangitis? (GPA)

A

Aka Wegener’s: granulomatous inflammation of vessels of respiratory tract (upper = rhinitis, sinusitis; lungs = pulmonary nodules, infiltrates) and kidneys (glomerulonephritis, renal failure). Initially have URTI symptoms; most common in middle age.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is eosinophilic granulomatosis with polyangitis (EPGA)?

A

Aka Churg Strauss;
Granulomatous inflammation of vessels with hypereosinophilia and eosinophilic tissue infiltration; frequent lung involvement (asthma, allergic rhinitis).
Other manifestations: coronary arteritis, myocarditis and neuropathy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Which antigens is EPGA associated with?

A

MPO or pR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Average age of EGPA onset?

A

EGPA/Churg Strauss onset = 40s.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is microangiopathic polyangitis?

A

Pauci-immune necrotising vasculitis affecting kidneys (necrotising glomerulonephritis), lungs (capillaritis and alveolar haemorrhage), skin.
Most common in middle age.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is polyarteritis nodosa?

A

Segmental, non-granulomatous necrotising inflammation. Unknown aetiology.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is Takayasu’s arteritis?

A

“Pulseless disease”: unequal peripheral pulses, chronic inflammation; most often affecting the aorta and its branches. Risk of aortic aneurysm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Who does Takayasu’s most often affect?

A

Usually young adults of Asian descent, F>M.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is Beurger’s disease and who does it affect?

A

Inflammation secondary to pathological clotting, affects small and medium size vessels of distal extremities, may lead to distal claudication and gangrene.

  • Most common in young Asian males
  • Most important etiologic factor is cigarette smoking.
24
Q

What is Behcet’s disease?

A

Leukocytoclastic vasculitis; multi system disorder presenting with ocular involvement (uveitis), recurrent oral and genital ulceration, venous thrombosis, skin and joint involvement.

25
Q

Who does Behcet’s commonly affect?

A

More common in Mediterranean and Asia; average age = 30s; M>F

26
Q

What is predominately cutaneous vasculitis subdivided into?

A
  • Drug induced vasculitis
  • Serum sickness reaction
  • Vasculitis associated with other underlying primary disease
27
Q

Which drugs are linked to predominately cutaneous vasculitis?

A
  • Allopurinol
  • Gold
  • Sulfonamides
  • Penicillin
  • Phenytoin
28
Q

Aetiology of predominately cutaneous vasculitis?

A
  • Drug exposure
  • Viral or bacterial infection
  • Idiopathic causes
29
Q

Which vessels are involved in predominately cutaneous vasculitis?

A

Small vessels; most frequently the post capillary vessels

30
Q

What are the signs and symptoms of primarily cutaneous vasculitis?

A
  • Palpable purpura +/- vesicles and ulceration
  • Urticaria
  • Macules
  • Papules
  • Bullae
  • Subcutaneous nodules
31
Q

Ix in ?Small vessel vasculitis?

A

-FBE/UEC/LFTs
-ESR / CRP
-RF
-ACCP
-ANA/ENA (if ANA +ve)
-Complement (C3/4)
Bx: vascular involvement (both arteriole and venule)

32
Q

Rx predominately cutaneous vasculitis?

A
  • Stop offending drug
  • Corticosteroids +/- immunosuppressives
  • Usually self limiting
33
Q

Which antibody is GPA most strongly associated with?

A

c-ANCA

34
Q

What are the classic features of GPA?

A
  • Necrotising granulomatous vasculitis of lower and upper respiratory tract
  • Focal segmental glomerulonephritis
35
Q

What are the criteria for GPA diagnosis?

A

2+ criteria for diagnosis:

  1. Nasal or oral involvement (inflammation, ulcers, epistaxis)
  2. Abnormal CXR (nodules, cavitations etc)
  3. Urinary sediment (protein, RBC casts)
  4. Biopsy of tissue involved (lungs = granulomas, kidneys = necrotising segment glomerulonephritis)
36
Q

What are the systemic S/S of GPA?

A
  • Malaise
  • Fever
  • Weakness
  • LoW
37
Q

What are the HEENT S/S of GPA?

A
  • Sinusitis or rhinitis, nasal crusting and bloody nasal discharge, nasoseptal perforation, saddle nose deformity
  • Proptosis
  • Hearing loss due to CNVIII involvement
38
Q

What are the pulmonary S&S of GPA?

A

Cough, haemoptysis

39
Q

CXR findings in GPA?

A
  • Pneumonitis
  • Lung nodules
  • Infiltrations
  • Cavitary lesions
40
Q

Treatment of GPA?

A
  1. Prednisone 1mg/kg/d PO 3-6/12
  2. +/- cyclophosphamide 2mg/kg/d PO 3-6/12
    Followed by
  3. High dose methotrexate (25mg PO weekly) or azathioprine (2g/kg/d PO OD)
    ALSO: consider biologics (rituximab, IVIg) and plasmapheresis
41
Q

What was demonstrated by the RAVE trial?

A

NEJM 2010: rituximab equivalent or superior to cyclophosphamide with less toxicity in GPA Rx

42
Q

What is PAN?

A

Systemic necrotising vasculitis of medium size vessels

43
Q

ANCA associated with PAN?

A

No. PAN is ANCA negative

44
Q

What is PAN often associated with?

A

Hep B +ve

45
Q

What is livedo reticularis?

A

Mottled, reticular pattern over skin

46
Q

Biopsy features of PAN?

A

Presence of granulocytes and / or mononuclear leukocytes in the artery wall

47
Q

Pathophysiology of PAN?

A

Focal, panmural necrotising inflammatory lesions in small and medium sized arteries; thrombosis, aneurysm or dilation at lesion site may occur. Healed lesions show fibrosis and endothelial proliferation –> may lead to luminal occlusion

48
Q

Rx PAN?

A

-Prednisone 1mg/kg/d PO
-Cyclophosphamide 2mg/kg/d PO
+/- antivirals for HepB clearance

49
Q

Ix in PAN?

A
  • Bloods: FBE/UEC/LFTs, ESR, pANCA, HepB serology
  • Imaging: angiography (beading)
  • Arterial biopsy
50
Q

Epidemiology of GCA?

A
  • Most frequent vasculitis
  • Patients >50y
  • 2F:1M
51
Q

Diagnostic criteria for GCA?

A

Diagnosed if 3+ present:

  1. Age >50
  2. New headache
  3. Temporal artery abnormality
  4. Elevated ESR
  5. Abnormal artery biopsy
52
Q

Biopsy features of GCA?

A

Mononuclear cell infiltration or granulomatous inflammation, usually with multinucleate giant cells

53
Q

S&S of GCA?

A
  • New onset temporal headache +/- scalp tenderness
  • Sudden painless loss of vision / diplopia
  • Tongue/jaw claudication
  • Polymyalgia rheumatica
  • Aortic arch syndrome
54
Q

How is GCA diagnosed?

A
  • Clinical suspicion
  • Increased ESR/CRP
  • Temporal artery Bx within 14d of steroids
55
Q

Rx of GCA?

A

High dose prednisone 1mg/kg for approximately 4/52

Cardiovascular (22 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions