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Year 4: MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Think [] if patients have multisystem features and are failing to respond to conventional therapies eg antibiotics for fevers, raised inflammatory markers and involvement of one or more vital organ system such as nerve, skin, kidney, lung

A

Think vasculitis if patients have multisystem features and are failing to respond to conventional therapies eg antibiotics for fevers, raised inflammatory markers and involvement of one or more vital organ system such as nerve, skin, kidney, lung

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2
Q

The clinical features of vasculitis depend on both the size of the vessels involved (e.g. large, medium or small) and the location of the vessels involved (e.g. kidney, skin or gut vessels).

However, there are certain clinical features that are strongly suggestive of a vasculitic process. These features include: [4]

A

Palpable purpura:
- vasculitis classically causes ‘palpable purpura’ that is often referred to as a ‘vasculitic rash’. Purpura are small (3-10 mm) red/purplish skin lesions that are non-blanching (i.e. do not go pale when pressed) and occur due to damaged blood vessels. Suggest cutaneous involvement of vasculitis

Constitutional symptoms:
- refers to features such as fever, weight loss, and fatigue. Non-specific and simply suggestive of a systemic process (e.g. infection, cancer, inflammatory disorder). Need to be considered in the context of other features

Asymmetrical neuropathies:
- damage to the small blood vessels that supply peripheral nerves can lead to peripheral neuropathy with sensory and/or motor features. Typically cause mononeuritis multiplex or an asymmetrical polyneuropathy

Unexplained bleeding:
- patients with unexplained haemoptysis or haematuria is suggestive of a vasculitic process affecting the small vessels of the pulmonary and renal vasculature. This may be seen with many small-vessel vasculitides

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3
Q

Describe the pathophysiology of polyarteritis nodosa (PAN) [3]

A

Inflammation of Medium-sized Arteries:
- The hallmark of PAN is necrotising inflammation of the medium-sized arteries, particularly the muscular and elastic type
- Immune Complex Deposition - in cases associated with HBV, immune complexes formed by the viral antigens and corresponding antibodies are thought to be deposited in the arterial walls, leading to inflammation and damage.

Ischaemia and Infarction:
- The inflammation and damage to the arterial wall can lead to stenosis or occlusion of the vessel, resulting in ischaemia and infarction of the downstream tissues.

Aneurysm Formation:
- The weakening of the arterial wall can lead to the formation of microaneurysms, which can rupture, causing haemorrhage.

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4
Q

Describe the clinical features of PAN [+]

A

ZtF:
* Renal impairment
* Hypertension
* Cardiovascular events
* Tender skin nodules

PM:
* fever, malaise, arthralgia
* weight loss
* hypertension
* mononeuritis multiplex, sensorimotor polyneuropathy
* testicular pain
* livedo reticularis
* haematuria, renal failure
* perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with ‘classic’ PAN

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5
Q

Investigations for PAN?

A

There is no diagnostic laboratory test for PAN.

Biopsy is performed on a clinically affected organ to confirm the diagnosis.

Arteriography (mesenteric or renal) can be used as an alternative to biopsy to confirm the diagnosis (to minimise bleeding risk). It can reveal aneurysms and irregular constrictions in the vessels.

Chest radiography may be obtained to exclude other forms of vasculitis, which have greater involvement in the lungs.

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6
Q

How would you differenitate PAN to atherosclerosis? [1]

A

Atherosclerosis can also cause infarctions in various organs, causing similar symptoms, with similar age of onset as PAN. They can be both differentiated on histology.

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7
Q

Tx of PAN?

A

Induction of Remission
* Corticosteroids - Prednisolone at a dose of 1mg/kg/day (maximum 60 mg/day) is usually recommended. Tapered
* Cyclophosphamide: For patients with severe PAN or organ-threatening disease, cyclophosphamide is usually added. The typical dose is 2 mg/kg/day orally or 15 mg/kg intravenously every 2-3 weeks.

Maintenance of Remission
* Azathioprine or Methotrexate: Once remission is induced, cyclophosphamide can be replaced with azathioprine (2 mg/kg/day) or methotrexate (15-25 mg/week) as maintenance therapy.
* Corticosteroids: Continue with a lower dose of corticosteroids and taper gradually.

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8
Q
A

Hep B

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9
Q
A

HTN

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10
Q
A

a 55-year-old man who has had fever and malaise for the past few weeks develops a common peroneal nerve palsy. Bloods show the presence of hepatitis B surface antigen

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11
Q
A

positive hepatitis B serology

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12
Q

On imaging, typical findings in PAN are [] and []

A

On imaging, typical findings in PAN are multiple aneurysms and irregular constrictions of arterial vessels.

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13
Q

Describe the typical presentation of Kawakasi disease [5]

A

Medium vessel vasculitis that presents in children

  • High grade fever lasting > 5 days (normally resistant to antipyretics)
  • Conjunctival injection
  • Bright cracked lips
  • Strawberry tongue
  • Cervical lymphadenopathy
  • Peeling, red palms
  • Coronary artery aneurysms
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14
Q

Management for Kawasaki disease? [3]

A

High dose aspirin
IV IG
Echo - used to screen for coronary artery aneurysms

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15
Q

Name 4 forms of small vessel vasculitis [4]

A

Henoch-Schonlein Purpura
Microscopic Polyangiitis
Granulomatosis with Polyangiitis
Eosinophilic Granulomatosis with Polyangiitis

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16
Q
A

A 2-year-old who has had a fever for the past 7 days is noted to have conjunctivitis, erythema and oedema of the hands and feet, cracked lips and a strawberry tongue

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17
Q
A

Bright red, cracked lips and strawberry tongue

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18
Q
A

child with fever, conjunctivitis, desquamating rash, cracked lips, strawberry tongue

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19
Q
A

red palms of the hands and the soles of the feet which later peel

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20
Q
A

Aspirin

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21
Q
A

coronary artery aneurysm

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22
Q
A

Intravenous immunoglobulin

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23
Q

Describe what is meant by Granulomatosis with polyangiitis? [1]

Which antibody is most commonly associated with patients of GPA? [1]

A

GPA:
- cANCA systemic vasculitis that affects small and medium vessels

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24
Q

Describe the basic pathophysiology of GPA

A

cANCA associated vasculitis - B cells produce due an autoimmune trigger

Causes vascular injury from cANCA deposits, and complexes formed after endothelial attachement causing release of more cytokines

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25
Q

Describe the clinical presentation of GPA

A

Patients typically present with:

URTI
- sinusitis
- saddle nose - due to nasal bridge collapse,
- otitis media
- nasal crusting

LRTI:
- Haemopytsis
- Dysopnea and cough
- Pleuritis
- Pulmonary infiltrates

Neurological
- Mononeuritis simplex
- Peripheral sensorimoto polyneuropathy
- Cranial neuropathy

Petechiaie, purpura

Glomerulonephritis

Consider granulomatosis with polyangiitis when a patient presents with ENT, respiratory and kidney involvement

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26
Q

A 48-year-old male presents to the GP as he has recently coughed up small amounts of blood on several occasions. He has also noticed his nose is ‘always blocked’ and has had a few episodes of nosebleeds. Upon questioning, he admits that his clothes feel a little looser but he has not weighed himself.

On examination, you notice a palpable rash on his lower legs.

Based on the most likely diagnosis, which antibodies are most likely to be found in this patient’s blood?

Anti-CCP
Anti-GBM
Anti-dsDNA
cANCA
pANCA

A

cANCA

This patient most likely has granulomatosis with polyangiitis (GPA) based on the history which includes ENT symptoms (rhinosinusitis and epistaxis), respiratory symptoms (cough and haemoptysis), and weight loss. Palpable purpura is also a common feature of GPA. cANCA is the antibody most commonly found in patients with GPA.

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27
Q

Describe the investigations used for GPA [3]

A

cANCA positive in > 90%, pANCA positive in 25%

chest x-ray:
- wide variety of presentations, including cavitating lesions

renal biopsy:
- epithelial crescents in Bowman’s capsule

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28
Q

What is the gold standard for dx GPA? [1]

How else do you investigate? [3]

A

Gold standard:
- histopathological confirmation of necrotising granulomatous inflammation in a biopsy sample from an affected organ, typically the lung or kidney.

Serology:
- cANCA
- proteinase 3 (PR3)

Abnormal urinary sediement:
- microscopic haematuria or red cell casts

Pulmonary abnormalities:
- nodules, fixed infiltrates or cavities observable on chest radiograph.

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29
Q

How do you differentiate GPA with eGPA?

A

eGPA presents more with asthma and eosinophilia

mononeuritis multiplex is more common in eGPA

In EGPA, pulmonary infiltrates are more transient vs GPA

Renal involvement in EGPA tends to be less severe than in GPA, presenting as mild proteinuria or microscopic haematuria rather than rapidly progressive glomerulonephritis.

30
Q

Describe the management plan for GPA in life/organ threatening disease [+]

A

Induction of remission:
- First-line therapy: Methylprednisolone IV for 3-5 days (followed by oral prednisolone) and cyclophosphamide for 3-6 months
- Second-line therapy: Methylprednisolone IV for 3-5 days (followed by oral prednisolone) and rituximab IV.

Maintenance of remission
* First-line therapy: Prednisolone and methotrexate (with folic acid).; Prednisolone and azathioprine
usually for 2 years following remission, but can vary.

31
Q

Describe the management plan for GPA in life/organ threatening disease [+]

A

Induction of remission:
- First-line therapy: Methylprednisolone IV for 3-5 days (followed by oral prednisolone) and methotrexate for 3-6 months
- Second-line therapy: Oral prednisolone and cyclophosphamide;
Oral prednisolone and rituximab.

Maintenance of remission
* First-line therapy: Prednisolone and methotrexate (with folic acid).; Prednisolone and azathioprine
usually for 2 years following remission, but can vary.

32
Q

Describe the clinical presentation of eGPA

A
  • asthma (late onset)
  • blood eosinophilia (e.g. > 10%)
  • paranasal sinusitis
  • mononeuritis multiplex
  • pANCA positive in 60%
33
Q

Lanham criteria

This criteria is formed of three components, which all need to be met for the diagnosis of EGPA: [3]

A
  • Asthma
  • Peak peripheral eosinophil count >1500 cells/microL (>1.5 x10^9/L)
  • Systemic vasculitis (with ≥2 extra-pulmonary organ involvement)
34
Q

Mx for eGPA

A

Induction:
- prednisolone at 0.5-1.0 mg/kg/day
- Methylprednisolone 1g daily for three days may be used for more extensive disease.
- Cyclophosphamide, which is an alkylating chemotherapeutic agent used in systemic inflammatory conditions, may be added for severe multi-system disease or an inadequate response to steroids.

Maintenance:
- This can be initiated after induction therapy to maintain disease remission over long periods of time. The choice of agents may include azathioprine or methotrexate, but there are many other options.

35
Q
A

Epithelial crescents in Bowman’s capsule

36
Q
A

rapidly progressive glomerulonephritis

37
Q
A

rapidly progressing GN

38
Q
A

saddle nose

39
Q
A

GPA

NB - eGPA has late onset asthma

40
Q
A

eGPA

41
Q
A

Churg-Strauss syndrome

42
Q
A

epithelial crescents in Bowman’s capsule

43
Q
A

rapidly progressive glomerulonephritis

44
Q

Microscopic Polyangiitis is associated with which antibody? [1]

The major autoantigens in microscopic polyangiitis are [2]

A

p-ANCA

The major autoantigens in microscopic polyangiitis are MPO and PR3.

45
Q

Clinical features of microscopic polyangiitis?

A

fever

palpable purpura

rapidly progressive glomerulonephritis
- raised creatinine, haematuria, proteinuria

Diffuse alveolar haemorrhage

Respiratory (25-55%): haemoptysis, pulmonary haemorrhage, pleural effusion, oedema

pANCA (against MPO) - positive in 50-75%
cANCA (against PR3) - positive in 40%

46
Q

How do you differentiate MPA and PAN? [2]

A

PAN:
- usually no lung involvement, strong link with Hepatitis B, only involves small to medium arteries (not venules or capillaries)

47
Q

Tx for severe MPA? [+]

A

Induction:
- 1st line: cyclophosphamide (CYC) with high dose steroids. Therapy is continued for 3-6 months then switched to less toxic maintenance once remission achieved
- Adjunct plasma exchange in patients with severe renal failure (creatinine >500 µmol/l) or life-threatening manifestations

Maintenance: for 24 months
- 1st line: low dose steroids with azathioprine (AZA) or MTX
- If patients refractory to AZA and MTX or contraindications to use: MMF or leflunomide

48
Q

Tx for non-severe MPA? [+]

A

Induction:
- High dose steroids with either methotrexate (MTX) or mycophenolate mofetil (MMF)

Maintenance: for 24 months
- 1st line: low dose steroids with azathioprine (AZA) or MTX
- If patients refractory to AZA and MTX or contraindications to use: MMF or leflunomide

49
Q

[] is a complication of cyclophosphamide treatment: 5% risk after 10 years and 16% risk after 15 years.

A

Bladder cancer: a complication of cyclophosphamide treatment: 5% risk after 10 years and 16% risk after 15 years.

50
Q

[Complication] patients have a nine-fold increased risk of mortality and a higher risk of relapse in MPA

A

Alveolar haemorrhage: patients have a nine-fold increased risk of mortality and a higher risk of relapse

51
Q

Define Henoch-Schonlein purpura (HSP) [1]

A

HSP is an acute immune complex-mediated small vessel vasculitis, characterised by the classic tetrad of rash, abdominal pain, arthritis/arthralgia, and glomerulonephritis

52
Q

Describe the pathophysiology of HSP

A

Type III immune complex-mediated hypersensitivity reaction, characterised by the tissue deposition of IgA-containing immune complexes within affected organs due to antigenic stimulus (such as infections, drugs, or toxins)

IgA antibody immune complexes deposits in vascular walls, stimulating complement activation

Immune complex deposition causes vessel necrosis which results in organ-specific symptoms.

53
Q

In HSP, immunofluorescence studies show [3] within the walls of involved vessels.

A

Immunofluorescence studies show IgA, complement component 3 (C3), and fibrin deposition within the walls of involved vessels.

54
Q

Describe the typical presentation of HSP

What is the typical tetrad? [4]
Other manifestations?

A

Usually preceded by a history of a preceding viral or bacterial upper respiratory tract infection (URTI)

Classic tetrad:
* Palpable purpura
* Arthritis/arthralgia
* Abdominal pain
* Renal disease

Other manifestations:
* Typically symmetrically distributed, non-blanching palpable purpura, especially on the lower legs, buttocks, knees and elbows.
* Arthralgias/arthritis (80%)
The knees and ankles are most often affected.
* Gastrointestinal symptoms (50-75%): N&V; bloody stools or melena. Intussusception and bowel infarction common
* Renal: causes IgA nephritis; symptomatic haematuria and/or proteinuria) to severe (i.e., rapidly progressive nephritis, nephrotic syndrome, and renal failure).

55
Q

Investigations for HSP:
Why would you perform a coagualation study and what would you expect to see? [2]

A

Should be normal in HSP.
Helps in ruling out other diagnoses such as thrombocytopenia

56
Q

How would you differentiate ITP from HSP? [3]

A

ITP:
- arthralgias and abdominal pain are uncommon.
- The platelet level is low in ITP but normal in HSP

57
Q

Describe the management of HSP [1]

A

The majority of patients with HSP recover spontaneously

58
Q

Give notable indications for hospitalisation in HSP [5]

A
  • Inability to maintain adequate hydration with oral intake
  • Severe abdominal pain
  • Notable gastrointestinal bleeding
  • Altered mental status
  • Renal involvement (elevated creatinine), hypertension, and/or nephrotic syndrome

TOMTIP NSAIDs should not be used in patients with active gastrointestinal bleeding or glomerulonephritis because of their effects on platelets and renal perfusion.

59
Q
A

IgA mediated small vessel vasculitis

60
Q
A

palpable purpuric rash over buttocks

61
Q

check that you have done temporal / giant cell arteritis in other notes

A
62
Q

Define Takayasu’s arteritis [1]

A

Takayasu’s arteritis is a rare, idiopathic, chronic inflammatory disease characterised by granulomatous inflammation of the aorta and its major branches.

63
Q

Which vessels does takayasu’s arteritis mainly affect? [2]

How does it affect these vessels? [1]

A

Aorta
Pulmonary arteries

Causes swelling and aneurysms OR become blocked and narrowed - causes reduction in pulses and BP in limb: pulseless disease

64
Q

Describe the typical patient of TA [1]

A

A stereotypical presentation of Takayasu’s arteritis often involves a young woman under the age of 40 who presents with systemic symptoms such as fever, malaise, night sweats, and weight loss. These non-specific symptoms may precede vascular manifestations by months or even years.

65
Q

Describe the clinical presentation of TA

A

Features depend on the organs affected.

Pulses
- Pulselessness: Diminished or absent pulses in one or more extremities is a hallmark feature.
- Blood pressure discrepancies between limbs

Claudication:
- Patients may experience limb claudication due to reduced blood flow, particularly in the upper extremities.

Bruits

Hypertension:
- Renal artery stenosis can lead to secondary hypertension. This is frequently resistant to conventional antihypertensive therapy.

Neurological and visual disturbances

Aortic regurgitation:
- Involvement of the ascending aorta can lead to dilation and subsequent aortic valve insufficiency.

66
Q

What is the preferred imaging modality for Takayusu arteritis? [1]

A

MRI Angiography (MRA):
- MRA provides detailed images of both the vascular lumen and surrounding structures without ionising radiation.

67
Q

Managment of TA? [2]

A

Inducing remission:
- Oral prednisolone (1mg/kg/day) is usually first line.
- Steroids should be given alongside low dose aspirin (75mg daily).

Immunosuppressive agents (utilised when patients relapse during steroid tapering)
* Methotrexate (given with folic acid).
* Azathioprine.
* Mycophenolate mofetil.
* Cyclophosphamide.

68
Q
A

intermittent claudication

69
Q
A

granulomatous thickening of the aortic arch

70
Q
A
71
Q
A

A 30-year-old woman from Singapore presents with headache, malaise and myalgia. On examination the blood pressure is 160/100 mmHg, the right radial pulse is difficult to palpate and a carotid bruit is noted

72
Q
A

absent radial pulse

Year 4: MSK (34 decks)

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