Vasculitises

Question 1

Temporal Giant Cell Arteritis

Answer 1

• Unilateral headache, jaw claudication, may lead to irreversible blindness (ophthalmic artery occlusion), assoc. with polymyalgia rheumatica
• Most commonly affects carotid artery branches
• Focal granulomatous inflammation, increased ESR
• Tx: high-dose corticosteroids

Question 2

Takayasu’s Arteritis

Answer 2

• Asian women <40yo has pulseless disease, arthritis/myalgias/skin nodules, ocular disturbances
• Granulomatous thickening of aortic arch, proximal great vessels, increased ESR
• Tx: corticosteroids

Question 3

Unilateral headache, jaw claudication in old person

Answer 3

Temporal GCA: may lead to irreversible blindness (ophthalmic artery occlusion); associated with polymyalgia rheumatica

• Most commonly affects carotid artery branches (temporal, ophthalmic)
• Focal granulomatous inflammation, increased ESR
• Tx: high-dose corticosteroids

Question 4

Asian women has weak upper extremity pulses, ocular disturbances

Answer 4

Takayasu’s Arteritis: Granulomatous thickening of aortic arch, proximal great vessels, increased ESR
*Tx: corticosteroids

Question 5

Polyarteritis nodosa

Answer 5

Associated with HBV; abdominal pain, melena, hypertension, neuro dysfunction, cutaneous eruptions, renal damage

• Immune complex-mediated, involve medium renal and visceral vessels (not pulmonary)
• Transmural inflammation of arterial wall with fibrinoid necrosis -> aneurysms and constrictions
• Tx: corticosteroids and cyclophosphamide

Question 6

Kawasaki’s disease

Answer 6

Mucocutaneous Lymph Node Syndrome: Asian child MI, Rupture

*Tx: IV IG and Aspirin!!!

Question 7

Beurger’s disease

Answer 7

Male smoker s gets intermittent claudication

• Risk of gangrene, digit amputation, superficial nodular phlebitis
• Tx: Stop smoking!

Question 8

Abdominal pain, melena, hypertension, neurodysfunction, cutaneous eruptions, renal damage in HBV+

Answer 8

Polyarteritis Nodosa (medium vessel vasculitis):

• Immune complex-mediated, involve medium renal and visceral vessels (not pulmonary)
• Transmural inflammation of arterial wall with fibrinoid necrosis -> aneurysms and constrictions
• Tx: corticosteroids and cyclophosphamide

Question 9

Asian child gets cervical lymphadenitis, strawberry tongue, hand-foot erythema, desquamating rash

Answer 9

Kawasaki disease aka Mucocutaneous LN Syndrome (medium vessel vasculitis):

• Risk of coronary aneurysm -> MI, rupture
• Tx: IV IG and Aspirin!!!

Question 10

Male smoker with a history of Raynaud’s gets intermittent claudication

Answer 10

Beurger’s disease (medium vessel vasculitis): Segmental thrombosing vasculitis

• Risk of gangrene, digit amputation, superficial nodular phlebitis
• Tx: Stop smoking!

Question 11

Microscopic Polyangiitis

Answer 11

Necrotizing small-vessel vasculitis involving lungs, kidneys, skin, with pauci-immune GN and palpable purpura
*No granulomas
*p-ANCA and MPO-ANCA +
Tx: Corticosteroids and Cyclophosphamide

Question 12

Wegener’s granulomatosis (with polyangiitis)

Answer 12

c-ANCA + small-vessel vasculitis
Triad: focal necrotizing vasculitis, necrotizing granulomas in lung and upper airway, necrotizing GN
*Upper respiratory: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, granulomas on hard palate
*Lower respiratory: hemoptysis, cough, dyspnea
*Renal: hematuria, red cell casts
CXR shows large nodular densities
Tx: Corticosteroids and Cyclophosphamide

Question 13

Churg-Strauss syndrome

Answer 13

Granulomatous, necrotizing vasculitis with eosinophilia

• Vasculitis in a young asthmatic
• p-ANCA and elevated IgE, eosinophilia
• Can also involve heart and kidneys

Question 14

Henoch-Schonlein Purpura

Answer 14

Triad: Palpable purpura on legs and butt, arthralgia, abdominal pain and melena

• IgA complex deposition -> vasculitis
• Associated with IgA nephropathy
• Most common childhood systemic vasculitis

Question 15

p-ANCA

Answer 15

pauci-immune GN +/-

• Microscopic polyangiitis (no granulomas, MPO-ANCA) or…
• Churg-Strauss syndrome (granulomatous, eosinophila, IgE, asthmatic)

Question 16

Necrotizing small-vessel vasculitis involving lungs, kidneys, skin, with pauci-immune GN and palpable purpura
*p-ANCA

Answer 16

Microscopic polyangiitis

• No granulomas
• Tx: Cyclophosphamide and Corticosteroids
• MPO-ANCA+ too

Question 17

Focal necrotizing vasculitis, necrotizing granulomas in lung and upper airway, necrotizing GN
*c-ANCA

Answer 17

Wegener’s granulomatosis (with polyangiitis):
*Upper respiratory: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, granulomas on hard palate
*Lower respiratory: hemoptysis, cough, dyspnea
*Renal: hematuria, red cell casts
CXR shows large nodular densities
Tx: Corticosteroids and Cyclophosphamide

Question 18

Granulomatous, necrotizing vasculitis with eosinophilia in an asthmatic
*p-ANCA

Answer 18

Churg-Strauss syndrome: small-vessel arteritis in a young asthmatic, with high IgE and eosinophilia
*Can also involve heart, GI, kidneys, cause wrist/foot drop

Question 19

Child with palpable purpura on legs and butt, arthralgia, abdominal pain and melena

Answer 19

Henoch-Schonlein purpura: IgA complex -> vasculitis, nephropathy

Question 20

Granulomatous vasculitis

Answer 20

Takayasu’s large-vessel (pulseless disease), Wegener’s granulomatosis (c-ANCA), Churg-Strauss syndrome (p-ANCA, IgE, eosinophila, asthmatic)