Vasculitis Flashcards

1
Q

What investigations would be done for presentation of:

  • swollen joint, warm to touch, swelling feels boggy, no tenderness and erythema
  • fatigue
  • asthma
A
  • Microscopy and cytology - help determine whether an inflammatory process is occurring
  • FBC, U+E, LFT, CRP, PV - inflammatory process
  • TFTs - hypothyroidism can present with joint pains and fatigue
  • Multi-system disease - screen for IA/CTD/vasculitis; RF, anti-CCP antibody, ANA + ANCA
  • Knee/chest X-RAY
  • Urine dip
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What types of large cell vasculitis are there?

A

Aorta > arteries

  • Giant cell arteritis
  • Takayasu’s arteritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What types of medium vessel vasculitis are there?

A

Arteries > arterioles

  • Kawasaki’s disease
  • Polyarteritis nodosa (PAN)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What types of small vessel vasculitis are there?

A

Capillaries > venues > veins

  • Microscopic polyangitis
  • Eosinophilic granulomatosis with polyangitis (Churg-Strauss disease)
  • Granulomatosis with polyangitis (Wegener’s)
  • HSP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is c-ANCA?

A

A positive c-ANCA reveals the anti-neutrophil cytoplasmic antibody is preferably targeting PR3, which if found mainly in the cytoplasm. Classically, Wegener’s/GPA is associated with c-ANCA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the key features of Wegener’s?

A
  • URT: nasal crusting, epistaxis, sinusitis
  • LRT: dyspnoea, haemoptysis, cavitating lesions in the lung on XR
  • Saddle nose deformity
  • Vasculitic rash, eye involvement e.g. proptosis
  • Cranial nerve involvement
  • Renal biopsy: epithelial crescents in Bowman’s capsule
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is p-ANCA?

A

MPO (myeloperoxidase) and this is found more in the peri-nuclear region. It is most commonly found in microscopic polyangitis and eosinophilic granulomatosis with polyangitis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the ACR criteria for EGPA?

A
  • Asthma
  • Eosinophilia of >10% in peripheral blood
  • Paranasal sinusitis
  • Pulmonary infiltrates
  • Histological confirmation of vasculitis with extravascular eosinophils
  • Mononeuritis multiplex or polyneuropathy
    4/6 is high specificity and sensitivity
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is a common manifestation of vasculitis?

A

Glomerulonephritis - blood and protein in urine. If there is blood, send it off for red cell casts. These would indicate nephritic syndrome - proof of multi-system involvement.

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

What is crescenteric glomerulonephritis?

A

Serious complication of ANCA-associated vasculitides. Can present with both nephrotic and nephritic features clinically. Light microscopy shows crescent formation in the glomerulus. Immunosuppression is required for this.

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

What is the treatment progression for vasculitis?

A
  1. Rapid induction of immunosuppression with steroids (prednisolone or methylprednisolone) with 2nd immunosuppressant e.g. cyclophosphamide or rituximab
  2. Once remission achieved, steroid-sparing agent e.g. methotrexate, azathioprine or MMF
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What do you need to consider with high dose steroids?

A
  • Gastro-protection
  • Bone protection
  • Screen for diabetes
  • Monitor BP/weight
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are common respiratory manifestations of EGPA?

A
  • Asthma
  • Haemoptysis
  • Pneumonitis
  • Allergic rhinitis
  • Paranasal sinusitis
  • Nasal polyps
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are common cardiac manifestations of EGPA?

A
  • Pericardial effusion
  • MI
  • Myocarditis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are common skin manifestations of EGPA?

A
  • Purpura skin nodules

- Livedo reticularis

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

What are common renal manifestations of EGPA?

A
  • Crescenteric glomerulonephritis
  • HTN
  • Renal failure
17
Q

What are common neurological manifestations of EGPA?

A
  • Mononeuritis multiplex

- Strokes

18
Q

What are common eye and gastro manifestations of EGPA?

A
  • Anterior uveitis
  • Mesenteric infarction
  • Bowel perforation
19
Q

How does GCA present?

A
  • > 60yrs
  • Temporal headache
  • Palpable temporal artery with absent pulsation
  • Jaw claudication
  • Scalp tenderness
  • Sometimes systemic - fever and lethargy
  • Visual disturbances: double vision, blurring, amaurosis fugax
20
Q

What is Kawasaki disease?

A
  • Commonly seen in children, important to catch due to serious complications such as coronary aortic aneurysms (echocardiogram screening)
  • Persistent fever (>5 days)
  • Lymphadenopathy
  • Strawberry tongue
  • Bilateral non-infective conjunctivitis, red eyes
  • Desquamating rash
  • Red palms and soles of feet which later peel
21
Q

What is Polyarteritis Nodosa?

A
  • Associated with HepB and more common in males
  • Medium sized arteries which lead to aneurysms which can accumulate along the vessels forming a “rosary sign”
  • Systemic: fever, malaise, arthralgia, weight loss
  • Hypertension
  • Mononeuritis multiplex, sensorimotor polyneuropathy
  • Testicular pain
  • Livedo reticularis
  • Haematuria, renal failure
  • P-ANCA
22
Q

What is Takayasu’s disease?

A
  • Large vessel vasculitis
  • Causes absent pulses in the branches of the aorta leading to lack of pulses in upper limbs
  • Can affect the kidneys
  • Systemic: malaise, headache
  • Unequal BP in upper limbs
  • Carotid bruit
  • Intermittent claudication
  • Aortic regurgitation
  • Management: steroids
23
Q

What is the management for GPA?

A
  • Steroids
  • Cyclophosphamide
  • Plasma exchange
  • Median survival - 8-9yrs
24
Q

What is the treatment for GCA?

A
  • High dose glucocorticoids should be started before the temporal artery biopsy comes back
  • Prednisolone if no visual loss
  • IV methylprednisolone if there is evolving visual loss
  • If there is vision changes they should be seen the same day by an ophthalmologist
  • Bone protection with bisphosphonates as long tapering course of steroids
25
Q

What medication can cause worsening of asthma symptoms in EGPA?

A

Leukotriene receptor antagonists e.g., montelukast