Vascular pathology

Question 1

Temporal (giant cell) arteritis

Answer 1

Large-vessel granulomatous vasculitis
Affects branches of the carotid artery
Most common vasculitis in adults >50

Presents with headache, visual disturbances, jaw claudication, and polymyalgia rheumatica

Tx: corticosteroids, prior to biopsy (due to risk of blindness)

Question 2

Takayasu arteritis

Answer 2

Large-vessel granulomatous vasculitis
Affects aortic arch at branch points
Classically affects young Asian females

Presents with visual and neurological symptoms, with weak/absent pulses in upper extremities

Tx: corticosteroids

Question 3

Polyarteritis nodosa

Answer 3

Medium-vessel necrotizing vasculitis
Affects all organs, except the lungs
Classically affects young adults
Associated with HBsAg+

Transmural inflammation with fibrinoid necrosis
“String-of-pearls” appearance on imaging due to microaneurysms

Tx: corticosteroids and cyclophosphamide

Question 4

Kawasaki disease

Answer 4

Medium-vessel vasculitis
Classically affects Asian children < 4 yo

Presents with fever, conjunctivitis, enlarged cervical lymph nodes, and erythematous rash of palms and soles

Coronary artery involvement is common

Tx: aspirin and IVIG

Question 5

Buerger disease

Answer 5

Medium-vessel necrotizing vasculitis involving digits
Highly associated with smoking
Segmental-thrombosing vasculitis

Tx: smoking cessation

Question 6

Wegener granulomatosis (granulomatosis with polyangitis)

Answer 6

Small-vessel necrotizing granulomatous vasculitis

Involves nasopharynx (sinusitis), lungs (bilateral nodular lung infiltrates), and kidneys (rapidly progressive GN)

c-ANCA + (anti-proteinase 3, targets neutrophils)

Tx: corticosteroids and cyclophosphamide

Question 7

Microscopic polyangitis

Answer 7

Small-vessel necrotizing vasculitis

Presentation similar to Wegener, except no nasopharyngeal involvement and no granulomas

p-ANCA + (anti-myeloperoxidase)

Tx: corticosteroids and cyclophosphamide

Question 8

Churg-Strauss Syndrome

Answer 8

Small-vessel necrotizing granulomatous vasculitis
Associated with asthma and peripheral eosinophilia
May cause mono- or poly-neuropathy

p-ANCA+

Question 9

Henoch-Schonlein Purpura

Answer 9

Small-vessel vasculitis due to IgA immune complex
Most common vasculitis in children
Usually occurs post-URI

Presents with palpable purport on buttocks and legs, GI pain and bleeding, and IgA nephropathy

Question 10

Behcet syndrome

Answer 10

Small-vessel vasculitis caused by immune complexes; often seen after viral infection.

Presents with aphthous ulcers, genital ulcers, uveitis.