Vasculitis

Question 1

What is vasculitis?

Answer 1

Histological term decribing inflammation in the vessel wall.

• Systemic inflammatory vasculitidies
  
  • Multi-system / organ involvement
  • Systemic symptoms and signs
• There are 2 features helpful in classifying vasculitis:
  
  • ANCA
  • Size of vessel

Question 2

What are the types of systemic vasculitis affecting small vessels?

Answer 2

• Microscopic polyangitis
• Granulomatosis and polyangitis
• Eosinophilic granulomatosis with polyangitis
• Henoch Schonlein Purpura

Question 3

What are the types of systemic vasculitis affecting medium vessels?

Answer 3

• Polyarteritis nodosa
• Kawasaki disease

Question 4

What are the types of systemic vasculitis affecting large vessels?

Answer 4

• Giant cell arteritis
• Takayasu’s arteritis

Question 5

What are the systemic clinical features of vasculitis?

Answer 5

• Weight loss
• Fatigue
• Night sweats
• Arthralgia
• Fever

Question 6

What are the clinical features of vasculitis found in the skin?

Answer 6

• Rash
• Purpura
• Ulcers

Question 7

What are the clinical features of vasculitis found in the kidneys?

Answer 7

• Haematuria
• Proteinuria
• AKI /CKD

Question 8

What are the neurological clinical features of vasculitis?

Answer 8

• Nerve involvement
  
  • Motor or sensory

Question 9

What are the respiratory clinical features of vasculitis?

Answer 9

Haemoptysis

Question 10

What are the ENT clnical features of vasculitis?

Answer 10

• Epistaxis
• Deafness
• Sinusitis

Question 11

Describe the epidemiology of polymyalgia rheumatica.

Answer 11

• Usually diagnosed in patients >65. Rare in patients <50.
• 20 / 100,000 >50years old.
• Peak incidence 70-80.
• Women:Men 2:1.
• Familial cases recognised.
• Geographical variation - higher in Scandanavian and Northern European countries.

Question 12

Describe the relationship between PMR and GCA.

Answer 12

• PMR more common than GCA (2-3x).
• PMR can precede or follow a diagnosis of GCA, or occur at the same time.
• 15% of PMR patients develop GCA.
• PMR seen in 50% of patients with GCA.

Question 13

Describe the pathophysiology of PMR.

Answer 13

• Unknown cause
  
  • HLA association: HLA-DR4
  • Proinflammatory cytokine interleukin (IL)-6 implicated
• Muscle is histopathologically normal, structures around the joint are mainly affected.
• ? infectious trigger.

Question 14

Describe the typical history of a patient presenting with PMR.

Answer 14

• Aching / stiffness in muscles / joints.
• Onset is often acute.
• Distribution:
  
  • Proximal and symmetrical
  • In shoulder / hip girdle
  • Can become distal involvement
• Timing - stiffness worse in the morning or after inactivity >30 minutes.
• Systemic features - fatigue, weight loss, depression.
• Can affect daily activities:
  
  • Getting out of a chair
  • Getting dressed

Question 15

What are the differentials for a patient presenting with ?PMR?

Answer 15

Question 16

What would you likely find on examination of a patient with PMR?

Answer 16

• Limited shoulder abduction
• Distal synovitis
• Reduced ROM
• Normal muscle strength

Question 17

What are the initial investigations to carry out in a patient with ?PMR?

Answer 17

• Bloods:
  
  • ESR / CRP - hallmarks of the disease; usually elevated.
  • FBC - normocytic / normochromic, thrombocytosis.
  • LFTs - raised ALP.
  • CK - normal.
  • Anti-CCP/ANA/RF - negative.
• Imaging (these are not required for diagnosis):
  
  • USS
  • MRI
  • PET

Question 18

How is PMR diagnosed?

Answer 18

• There are no formal diagnostic criteria.
• Diagnosis is based on typical clinical features and lab findings.

Question 19

Describe the treatment for PMR.

Answer 19

• Steroids are the mainstay of treatment - prompt response.
• ADULT (by mouth)
  
  • ​10-15mg daily until remission of disease activity; maintenance 7.5-10mg daily, reduce gradually to maintenance dose.
  • Many patients require Rx for at least 2 years and in some patients it may be necessary to continue long term low-dose corticosteroid Rx.
• Prednisolone
  
  • High dose
  • Slow tapering
  • Careful monitoring
• Steroid-sparing agents
  
  • Methotrexate

Question 20

Describe the pathology of giant cell arteritis (GCA).

Answer 20

• AKA cranial arteritis or temporal arteritis.
• Symptoms and signs of GCA result from involvement of the cranial branches of arteries that originate from the aortic arch.
• The name reflects the type of inflammatory cell involved.

Question 21

Describe the typical history of a patient with GCA.

Answer 21

• Headaches - temporal.
• Tenderness of the scalp or temples - unable to brush hair.
• Diplopia / vision loss - transient painless monocular visual loss.
• Dizziness or problems with coordination and balance.
• Jaw claudication.

Question 22

Describe the likely examination findings of a patient with GCA.

Answer 22

Question 23

Which initial investigations should be carried out in a patient with ?GCA?

Answer 23

• Bloods
  
  • ESR / CRP - hallmark of the disease, usually elevated.
  • FBC - normocytic / normochromic, thrombocytosis.
  • LFTs - raised ALP.
• Imaging
  
  • USS - increased diameter of the superficial temporal artery and hypoechoic wall thickening (halo sign).
• Temporal artery biopsy
  
  • Infiltration of T lymphocytes, macrophages and giant cells in the vessel wall.
  • Granulomatous inflammation of intima and media.
  • Breaking up of internal elastic lamina with giant cells and plasma cells.

Question 24

Describe the treatment for GCA.

Answer 24

• Corticosteroid
• FOR ADULT (by mouth)
  
  • ​40-60mg daily until remission of disease activity; the higher dose being used if visual symptoms occur; maintenance 7.5-10mg daily, reduce gradually to maintenance dose.
  • Many patients require treatment for at least 2 years and in some patients it may be necessary to continue long term low-dose corticosteroid treatment.

Question 25

What is the prognosis for GCA patients?

Answer 25

• 18-24 months of steroid often required.
• Relapse rate of 25-50%.