Vasculitis

Question 1

Define polymyalgia rheumatica (PMR)

Answer 1

A syndrome of sudden (<2wk) onset severe pain and stiffness in a limb girdle pattern (shoulders, neck, hips, and lumbar spine). N.B. no weakness Symptoms are worse in the morning, with duration >30 minutes. Always occurs in over 50yrs.

Question 2

Name 2 differential diagnoses of Polymyalgia rheumatica (PMR)

Answer 2

Polymyositis: proximal muscle ache and weakness Myopathy: weakness, but no pain or stiffness PMR: proximal morning pain and stiffness, no weakness

Question 3

Describe the investigations of Polymyalgia rheumatica

Answer 3

Raised ESR and/or CRP* are hallmark Serum ALP and GGT may be raised as markers of acute phase response Anaemia is often present

Question 4

What is the management of Polymyalgia rheumatica?

Answer 4

Prednisolone 10-15mg/d PO + PPI Improvement should be seen within 2 weeks* R/V at 8 weeks

Question 5

Define Giant cell arteritis

Answer 5

• Inflammatory granulomatous arteritis of temporal arteries
• Associated with polymyalgia rheumatica (50%).
• Typically seen in women over 50

Question 6

Describe the clinical features of Giant cell arteritis

Answer 6

• Jaw and/or tongue claudication
• Severe headaches
• Scalp/temple tenderness e.g. when combing hair
• Tender swollen temporal or occipital arteries
• Changes in vision: curtain drop (amaurosis fugax)

Question 7

What will investigations of Giant cell arteritis show?

Answer 7

• Raised ESR
• Very high CRP
• Temporal artery biopsy (within 1 week)
  
  • Granulomatous infiltration
  • Giant cells

Question 8

Why must a temporal artery biopsy be >1cm when investigating Giant cell arteritis?

Answer 8

Giant cell arteritis can occur in a discontinuous pattern, so a long biopsy is needed to ensure it is not missed.

Question 9

Why is Giant cell arteritis a medical emergency?

Answer 9

It may cause sudden, painless vision loss if the opthalmic artery is involved.

Start treatment immediately if suspected

Question 10

Describe the diagnostic criteria for giant cell arteritis

Answer 10

3 of 5 of the following:

• Granulomatous inflammation
• Increased CRP and ESR
• Aged over 50
• New headache
• Temporal pain

Question 11

Outline the management of Giant cell arteritis

Answer 11

• Prednisolone
  
  • Uncomplicated (no claudication): 40mg
  • Complicated: 60mg
• PPIs
• Bisphosphonates

Wean steroids when symptoms resolve and ESR falls

Question 12

Define Takayasu’s arteritis

Answer 12

A rare large vessel arteritis more commonly seen in Japan. It involves the aortic arch and other major arteries.

Question 13

What are the characteristic features of Takayasu’s arteritis?

Answer 13

Absence of peripheral pulses HTN

Question 14

What is the treatment of Takayasu’s arteritis?

Answer 14

Corticosteroids for symptoms Surgical bypass of affected areas

Question 15

Define Classical polyarteritis nodusa (PAN)

Answer 15

A rare medium vessel vasculitis that typically occurs in middle-aged men. Accompanied by severe systemic maifestations.

Question 16

Describe the clinical features of Classical polyarteritis nodusa

Answer 16

Initial: Fever, malaise, weight loss, myalgia Acute organ infarction: -Neuro: Mononeuritis multiplex -GI: Pain mimicking acute cholecystitis (continuous RUQ), pancreatitis (epigastric/central radiating to back), or appendicitis (Central to RIF). GI haemorrhage -Renal: Haematuria and proteinuria -Cardiac: MI and heart failure -Skin: SC haemorrhage and gangrene. Livedo reticularis

Question 17

What is the treatment of Classical polyartheritis nodusa?

Answer 17

Corticosteroids + immunsuppressive drugs e.g. Azathioprine

Question 18

Define Kawasaki’s disease

Answer 18

An acute systemic medium vessel vasculitis supposedly triggered by infection, that mainly affects children under 5yr. Commonly seen in Japan.

Question 19

Describe the diagnostic criteria of Kawasaki’s disease

Answer 19

Persistent fever (>5 days) plus 4 of the following: Bilateral conjunctival congestion after 2-4days Dry red lips and oral cavity Acute cervical lymphadenopathy Polymorphic rash Red oedematous palms and soles

Question 20

Outline the management of Kawaski’s disease

Answer 20

Single high-dose IV immunoglobin to prevent CAD, followed by Aspirin 200-300mg daily

Question 21

Describe the clinical features of small-vessel vasculitides (ANCA positive)

Answer 21

Small-vessel vasculitides (ANCA positive) include GPA. eosinophilic GPA, and microscopic polyangiitis. They show either a c-ANCA (GPA) or p-ANCA (eGPA or microscopic polyangiitis) pattern. Respiratory tract and kidneys are frequently involved. ESR is often markedly elevated (>100)

Question 22

Describe the investigations of Granulomatosis with polyangiitis

Answer 22

c-ANCA pattern and granulomas on biopsy CXR: Multiple cavitating nodules, consolidation, ground glass opacification

Question 23

Who typically gets Granulomatosis with polyangiitis?

Answer 23

Elderly Caucasian

Question 24

Outline the presentation of Granulomatosis with polyangiitis

Answer 24

URT: Bloody nasal discharge with crusting Saddle-nose deformity Sinusitis Otitis media Diffuse alveolar haemorrhage: Cough Dyspnoea Pleurisy Glomerulonephritis

Question 25

What is the treatment for Granulomatosis with polyangiitis?

Answer 25

Corticosteroids and cyclophosphamide Rituximab Methotrexate

Question 26

How does microscopic polyangiitis differ from GPA?

Answer 26

GPA is associated with c-ANCA, features granulomas on biopsy. Microsopic is associated with p-ANCA, no granulomas on biopsy.

Question 27

Describe the presentation of eosinophilic GPA

Answer 27

Presents in early adulthood with allergic rhinitis, difficult to control asthma, and peripheral blood eosinophilia. Later systemic involvement: Skin: Tender SC nodules, petechiae, purpura Neuro: Mononeuritis multiplex Heart Kidneys GI

Question 28

How is eosinophilic GPA investigated?

Answer 28

CXR: migratory patchy opacification, nodules, effusion Biopsy: Eosinophilic infiltration, small-vessel vasculitis p-ANCA pattern

Question 29

What is the treatment of eosinophilic GPA?

Answer 29

Corticosteroids Immunosuppressive agents if severe

Question 30

Define Goodpasture syndrome

Answer 30

Autoimmune disease that damages the basement membranes in the lungs and kidneys.

Question 31

Describe the presentation of Goodpasture syndrome

Answer 31

Characteristic triad of: Pulmonary haemorrhage Glomerulonephritis Presence of antibodies against basement membrane antigens

Question 32

How is Goodpasture syndrome treated?

Answer 32

Plasmapheresis to remove circulating antibodies Prednisolone + cyclophosphamide to prevent further antibody production