Systemic inflammatory vasculitis

Question 1

Polymyalgia rheumatica (PMR) symptoms

Answer 1

sudden onset severe pain and stiffness of shoulders, hips, neck, lumbar spine, worse in morning
elderly women > 50

Question 2

PMR treatment

Answer 2

prednisolone low dose (10-15 mg) reduce gradually in weeks / months,
no NSAIDs

Question 3

Giant cell arteritis (GCA) patho

Answer 3

inflammatory granulomatous arteritis of large cerebral arteries
disease of elderly > 50 years
associated with PMR

Question 4

GCA symptoms

Answer 4

severe headaches, tenderness scalp or temple, claudication jaw when eating, tenderness & swelling temporal or occipital artery, sudden loss vision (ophthalmic artery)

Question 5

GCA treatment

Answer 5

prednisolone high dose (60-100 mg) reduce gradually

Question 6

Takayasu’s arteritis

Answer 6

granulomatous inflammtion of aorta and its major branches

Question 7

Polyarteritis nodosa (PAN) patho

Answer 7

rare, middle-aged men

fibrinoid necrosis of vessel walls with microaneurysms, thrombosis, infarction

Question 8

PAN treatment

Answer 8

corticosteroids in combi with immunosuppressants (azathioprine)

Question 9

Kawasaki’s disease symptoms

Answer 9

acute systemic vasculitis, children < 5 years
fever > 5 days, bilateral conjunctival congestion, dryness & redness lips and oral cavity, enlarged lymph nodes, redness & oedema palms and soles

Question 10

Treatment kawasaki’s

Answer 10

single-dose of high-dose IV immunoglobulin

after acute phase: aspirin

Question 11

Small vessel vasculitis: ANCA associated diseases?

Answer 11

Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis

Question 12

Small vessel vasculitis: immune complex diseases (ANCA negative)?

Answer 12

Anti-GBM disease
IgA vasculitis (Henoch-Schönlein)
Cryoglobulinaemic vasculitis
Hypocomplementaemic vasculitis
Cutaneous leucocytoclastic vasculitis (hep C)

Question 13

Granulomatosis with polyangiitis (GPA)

treat with immunosuppressants

Answer 13

ears and upper respiratory tract,
bloody nasal discharge, crusting, destruction, sinusitis, otitis media
respiratory –> cough, dyspnoea, pleuritic chest pain

Question 14

Eosinophilic granulomatosis with polyangiitis (EGPA)

treat with corticosteroids + immunosuppressants

Answer 14

allergic rhinitis, asthma difficult to control, peripheral blood eosinophilia
X-ray shows migratory patchy opacities

Question 15

Microscopic polyangiitis (MPA)

Answer 15

similar to GPA, abscence of granuloma formation