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Musculoskeletal and Orthopedics > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Define vasculitis?

A

Vasculitis is an inflammatory disorder of blood vessel walls, causing destruction (aneurysm/rupture) or stenosis.

Can affect vessels of any organ and presentation depends on which organs are involved

Can be primary or secondary to other diseases e.g. SLE, RA, hep B and C, HIV

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2
Q

what are the 3 types of large vessel vasculitis?

A

giant cell arteritis

polymylagia rheumatica

takayasu’s arteritis

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3
Q

what is the target artery, key info and classic presentation of giant cell arteritis?

A

target artery- carotid artery and branches

key info

  • affects elderly
  • associated with PMR
  • Treat urgently with oral predinsiolne

Classic presentation

  • Temporal artery- headache, scalp tenderness
  • Facial artery- jaw claudication
  • opthalmic artery- loss of vision
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4
Q

what is the target artery, key info and classic presentation of polymylagia rheumatica?

A

target artery- unknown

key info

  • associated with GCA
  • treat with oral steroids

Classic presentation

  • bilateral pain in morning
  • stiffness in pelvic, neck and shoulder girdle muscles
  • no weakness
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5
Q

what is the target artery, key info and classic presentation of takayasu’s arteritis?

A

target artery- branches of aortic arch

key info- affects asian women ( 20-40 yrs)

classic presentation

  • inflammatory phase: FLAWS
  • pulseless phase: weak/absent UL/LL pulses, claudication
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6
Q

state 2 medium vessel vasculitis?

A

polyarteritis nodosa

kawasaki

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7
Q

what is the target artery, key info and classic presentation of polyarteritis nodosa?

A

target artery: skin, renal and mesenteric

key info

  • associated with Hep B
  • Rosary sign on renal angiogram

classic presentation

  • consitiutional upset
  • skin rash
  • abdominal pain
  • GI bleed
  • hypertension
  • renal failure
  • peripheral neuropathy
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8
Q

what are the two divisions of small vessel vasculitis?

A

ANCA positive and ANCA negative

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9
Q

what are the 3 types of ANCA positive vasculitis?

A

granulomatosis with polyangitis ( Wegner’s)

Eosinophilic granulomatosis with polyangitis

microscopic polyangitis

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10
Q

what is the ANCA, key info and classic presentation of granulmatosis with polyangitis ( Wegener’s)?

A

cANCA

Key info

  • Saddle node
  • can cause cavitating lung lesions

Classic presentation

  • upper resp tract: nosebleeds, rhinitis , sinusitis
  • lower resp tract: haemoptysis
  • kidneys: glomerulonephritis
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11
Q

what is the ANCA, and classic presentation of microscopic polyangitis?

A

pANCA

Rapidly progressing glomeulonephritis

pulmonary haemorrhage ( haemoptysis)

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12
Q

what is the ANCA, key info and classic presentation of eosinophilic granulomatosis with polyangitis?

A

pANCA

Triphasic

allergic phase- asthma/ rhinitis

eosinophillic phase- high eosinophills-> tissue damage

vasculitic phase- widespread organ damage and death

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13
Q

what are the 3 types of ANCA negative small vessel vasculitis?

A

Hennoch schonlein purpura

Bechets disease

Goodpasture’s syndrome

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14
Q

What are the key features and classic features of Hennoch Schonlein purpura?

A

key info

  • affects 3-15 yr old children
  • IgA vasculitis

Classic presentation- TRIAD

  • purpura rash on buttocks and extensors of lower limbs
  • abdominal pain
  • arthralgia of larger joints
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15
Q

What are the key features and classic presentaiton of Bechet’s disease?

A

key info

  • more common in Greek and turkish people

classic presentation- TRIAD

- recurrent oral ulcers

  • genital ulcers
  • uveitis
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16
Q

what are the key features and classic presentation of Goodpasture’s syndrome?

A

key info

  • anti-GBM antibodies

Classic presentation- TRIAD

  • glomerulonephritis-> renal failure
  • haemotysis ( Pulmonary haemorrhage
  • anti- GBM anitbodies
17
Q

what are the risk factors for vasculitides?

A

Hepatitis B- polyarteritis nodosa

Hepatitis C - mixed essential cryoglobulinaemia

pANCA- microscopic polyangiitis + Churg-Strauss ( eosinophilic granulomatosis with polyangitis)

c-ANCA- Wegner’s granulomatosis

Hx of asthma, allergic rhinitis or sinusitis – Churg-Strauss

18
Q

outline the aetiology of vasculitides?

A

Suggested autoimmuneorigin

WBCs mistake self-antigens on endothelial cells as foreign antigens due to molecular mimicry and hence cause damage.

Immune complex deposition in the walls of blood vessels leads to inflammation

In other cases, WBCs damage cells near the endothelial cells and as a result, endothelial cells get damagedindirectly. This tends to occur in the small vessels.

The vessel wall damage leads to three things:

  • The damage exposes underlying collagen and TF increasing coagulation
  • The weaker walls also make aneurysmsmore likely
  • Healing of the walls causesscarring fibrosis which makes the vessels stiffer and narrower

=> reduced blood flow to organs downstream causing ischaemia

19
Q

summarise the epidemiology of vasculitides?

A

RARE

Takayasu’s arteritisis most common in JAPANESE FEMALESand women < 40

GCA is most common in women over 55

Granulomatosis with polyangiitis most common in middle aged men

Kawasaki most common in children <5, especially boys

20
Q

what is the general rule for the signs and symptoms seen in vasculitides?

A

Large vessel vasculitides have classic clinical patterns based on the vessels affected (e.g. GCA and loss of vision/headache)

Medium and small vessel vasculitides are characterised by multiorgan involvement and have less specific clinical features

21
Q

what are the features of GCA?

A

loss of vision, jaw claudication, headache, scalp tenderness

22
Q

What are the features of Takasayu’s?

A

Takayasu: affects arteries branching off from the aortic arch.

If it affects parts supplying upper extremities, you get weak or no pulse.

If it affects supplying the head, you get visual and neurological symptoms

23
Q

what are the feartures of kawasaki?

A

Affects coronary arteries

Can cause MI

Causes conjunctivitis, rash, lymphadenopathy, strawberry tongue, swollen hands and feet, and fever

24
Q

what are the features of polyarthritis nodosa?

A

This occurs when immune cells directly damage endothelium confusing it with hep B cells

Association with hepatitis B

Causes transmural inflammation of entire blood vessel wall, which leads to necrosis and then fibrosis. The weak fibrosis leads to fibrotic aneurysmsalong the vessel wall

Can occur anywhere causing organ ischaemia

If it affects renal arteries, it causes hypertension

If it affects mesenteric arteries, causes mesenteric ischaemia and severe abdo pain

Signs: typical systemic features, skin rash and punched out ulcers, microaneurysms, thrombosis, infarctions, hypertension, testicular pain

25
Q

describe the aetiology of ANCA positive small vessel vasculitis?

A

For ANCA positive diseases, B cells mistakenly target their antibodies (called ANCA) against granules made by neutrophils

This causes neutrophils to release oxygen free radicals that cause indirect damage to nearby endothelial cells

ANCAs are usually IgG and there are two types: cANCA and pANCA

26
Q

what are the features of granulomatois with polyangitis ( Wegener’s)

A
  • Affects nasopharynx, lungs and kidneys
  • Nasopharynx: chronic pain from sinusitis, bloody mucous and nasal discharge, saddle nose shape
  • Lungs: breathing difficulties, haemoptysis, chest pain, etc
  • Kidneys: glomerulonephritis, decreased urine production, hypertension
  • cANCA
27
Q

what are the features of microscopic polyangitis?

A

Very similar to Wegener’s but does not affect nasopharynx, only kidneys and lungs

Also pANCAassociated rather than cANCA

28
Q

what are the features of Eosinophilic granulomatosis with polyangiitis (Churgg-Straus syndrome)

A
  • Associated with pANCAs
  • Affects nasopharynx, kidneys, lungs, GI, skin, nerve and heart
  • Nasal discharge or stuffiness – purulent or bloody with facial pain
  • Associated with asthma(so WHEEZE), allergic rhinitis or sinusitis
  • Has high eosinophil count
29
Q

what are the features of Henoch- schonlein purpura?

A
  • This is not ANCA associated, but rather due to high IgA antibodies in the blood which directly target endothelial cells due to molecular mimicry
  • Purpura, arthritis, gut symptoms (abdominal pain), glomerulonephritis (haematuria), IgA deposition
  • The purpura is palpable – can feel it raised above skin due to fibrosis
30
Q

what are the investigations for vasculitides?

A

Bloods

Autoantibodies - e.g. cANCA in Wegner’s

HBV serology – for polyarteritis nodosa

Urine - haematuria, proteinuria, red cell casts (if glomerulonephritis)

Microscopic haematuria is common for Wegner’s

PFTs – check for asthma esp for Churg-stauss

CXR - diffuse, nodular or flitting shadows, atelectasis

Biopsy - renal, lung, temporal artery – in GCA, but note that GCA is segmental so biopsy requires a long section

Echo – to see if there is pericardium involvement

Angiography - to identify aneurysms

31
Q

interpret the results in vasculitides?

A

FBC - normocytic anaemia, high platelets, high neutrophils

Eosinophil count – increased with Churg-strauss

High ESR/CRP

Urea and Creatinine high for renal failure

Complement – may be low in polyarteritis nodosa

LFTs – mild elevation is common esp in polyarteritis nodosa

Musculoskeletal and Orthopedics (17 decks)

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