Vasculitis

Question 1

What is systemic vasculitis and its cause?

Answer 1

It is an autoimmune disease affecting lots of blood vessels which therefore affect many organs.
It has a mixture of genetic factors and environmental factors (exposure or infection)

Question 2

What are some types of large vessel vasculitis?

Answer 2

• Takayasu Arteritis,
• Giant Cell Arteritis

Question 3

What are some types of medium vessel vasculitis?

Answer 3

• Polyarteritis nodosa,
• Kawasaki Disease,

Question 4

What are some types of immune complex small vessel vasculitis?

Answer 4

• Cryoglobulin vasculitis,
• IgA vasculitis (Henoch-Schonlein)
• Hypocomplementemic urticarial vasculitis

Question 5

What are some types of ANCA-Associated small vessel vasculitis

Answer 5

• Microscopic Polyangiitis,
• Granulomatosis with polyangiitis (Wegner’s)
• Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

Question 6

What is the presentation of Giant Cell Arteritis

Answer 6

• Prevalence increases with advancing age.
• Presents with a constant temporal headache and temporal artery thickening/tenderness with little relief from analgesia.
• Visual symptoms
• Scalp tenderness
• Jaw claudication (occurs when eating/talking)
• Polymyalgia rheumatic symptoms (pain around shoulders, neck and hips) it has a strong association with polymyalgia rheumatica,
• Constitutional upset

Question 7

What are the complications of giant cell arteritis?

Answer 7

1. Irreversible visual loss (sudden painless loss of vision)
2. Large vessel vasculitis which can cause vascular stenosis and aneurysms.
3. Cerebral vascular accident.

Question 8

Explain the diagnosis of giant cell arteritis

Answer 8

1. Clinical presentation (Typical headache with associated features, appropriate age with no alternative diagnosis.)
2. Clinical exam findings (temporal artery asymmetry, thickening and loss of pulsatility)
3. Acute phase response (Raised CRP/ESR with no signs of infection)
4. Gold standard is temporal artery biopsy to look for inflammation. However temporal artery USS, MRI and PET CT can also be used.

Question 9

What is the treatment for suspected giant cell arteritis?

Answer 9

If strong clinical suspicion treat while you await results with prednisolone (1mg/kg/day) and 75mg Aspirin (unless contraindicated)

Question 10

What is the treatment of confirmed giant cell arteritis?

Answer 10

Maintain 60mg of prednisolone for 1 month then aim to taper to 15mg by 12 weeks with discontinuation by 12-18months.
If patients has relapsing disease on steroids then use: Mycophenolate, methotrexate or tocilizumab (anti IL6)

Question 11

What is the differential diagnosis for cutaneous (small vessel) vasculitis/leucocytoclastic vasculitis and an example?

Answer 11

• Differentials include idiopathic, drugs, infections (meningococcal), secondary to other autoimmune disease, malignancy.
• Example of a cutaneous/leucocytoclastic vasculitis is Henoch Schonlein Purpura,

Question 12

What is the presentation of Henoch Schonlein Purpura

Answer 12

• Commonly occurs in children but can occur in adults. It is a self limiting illness lasting 4-16 weeks. Commonly triggered by streptococcal sore throat.
• It presents with: purpuric rash on buttocks and thighs, urticarial rash, confluent petechiae, ecchymoses (bruise), ulcers and arthralgia/arthritis

Question 13

What are the complications of Henoch Schonlein purpura

Answer 13

• Renal: IgA nephropathy
• Urological: Orchitis
• CNA very rare to get complications.
• GI disease and renal disease are the most significant complications

Question 14

Explain the diagnosis and treatment of Henoch Schonlein Purpura

Answer 14

Diagnosis - Exclude secondary causes via history (any recent upper respiratory infections?), examination, immunology and virology. Assess the extent of involvement via urinalysis/urine PCR
Treatment - Often none but may use steroids with complications (testicular torsion, GI disease, Arthritis)

Question 15

What is the classic triad of Henoch Schonlein Purpura?

Answer 15

Purpuric rash on extensors of lower limbs, abdominal pain and arthritis.

Question 16

What is the classic triad of symptoms for granulomatosis with polyangiitis vasculitis?

Answer 16

• URT involvement: Chronic sinusitis, epitaxis and saddle node deformity.
• LRT involvement: Cough, haemoptysis and pleuritis (cavitation’s on CXR)
• Pauci-immune glomerulonephritis: Haematuria and proteinuria (SO DO URINE DIP!)
• Also get constitutional symptoms, eg fatigue, weight loss, fever, myalgia or arthralgia

Question 17

What are organ specific features of ANCA vasculitis?

Answer 17

• Ulceration throughout oral mucosa,
• Foot drop,
• Pericarditis,
• Glomerulonephritis,
• In lungs there can be cavities, bleeds and infiltrates.
• On skin there can be nodules on elbow and purpura.
• In the eye there can be pseudotumours and conjunctivitis

Question 18

What is the presentation of eosinophilic granulomatosis with polyangiitis (Churg-strauss) vasculitis?

Answer 18

• Asthma,
• Nasal polyposis
• Constitutional symptoms,
• Eosinophilia >10% in peripheral blood,
• Paranasal infiltrate,
• Pulmonary infiltrates,

Question 19

What is microscopic polyangiitis and its presentation

Answer 19

• Common small vessel vasculitis with renal disease, pulmonary disease and pANCA directed against myeloperoxidase
• Symptoms include: Constitutional symptoms, kidney inflammation (necrotising glomerulonephritis), skin lesions (purpura on lower limbs), peripheral nerve damage and lung involvement (alveolar haemorrhage and haemoptysis)

Question 20

What are the auto-antibodies detected in ANCA vasculitis and what are the methods of detection?

Answer 20

cANCA - targeted against PR3
pANCA - targeted against MPO
However not all ANCA vasculitis has positive ANCA (anti-neutrophil cytoplasmic antibodies).
It can be detected via indirect immunofluorescence or ELISA

Question 21

What autoantibodies are associated with different types of ANCA vasculitis?

Answer 21

cANCA with PR3 suggests granulomatosis polyangiitis
pANCA with MPO suggests microscopic polyangiitis or eosinophilic granulomatosis polyangiitis.

Question 22

What are the treatment options to induce remission in vasculitis?

Answer 22

Prednisolone and one of the following: Cyclophosphamide (risks of infection, cytopenia, malignancy and infertility) or Rituximab (no risk of infertility) which are excellent or methotrexate or mycophenolate.

Question 23

What are the treatment options to maintain remission in vasculitis?

Answer 23

Use of lower toxicity drugs for a prolonged period of time. Examples include: Azathioprine, methotrexate or rituximab

Question 24

What is seen on a temporal artery biopsy in giant cell vasculitis?

Answer 24

Multinucleated giant cells