Vasculitides (are da worst)

Question 1

Giant cell temporal arteritis demographics

Answer 1

Elderly females

Question 2

GCA symptoms

Answer 2

Unilateral headache, jaw claudication, can cause unilateral/irreversible blindness

Question 3

MOA of blindness in GCA

Answer 3

Opthalmic artery occlusion (disease affects branches of carotid a. most frequently)

Question 4

GCA association

Answer 4

Polymyalgia rheumatica

Question 5

Histology of GCA

Answer 5

Granulomatous inflammation along the internal elastic lamina –> focal destruction and fibrosis of vessel wall –> wall thickening, luminal narrowing

Question 6

Takayasu arteritis demographic

Answer 6

Asian females <40yrs

Question 7

Takayasu arteritis signs

Answer 7

Weak upper extremity pulses, fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances

Question 8

Takayasu arteritis histology

Answer 8

Mononuclear inflammation w/ giant cells through full thickness of vessel wall (aortic arch and branches), w/ fibrosis, assc. w/ wall thickening and luminal narrowing

Question 9

Granulomatosis w/ polyangiitis test

Answer 9

C-ANCA (anti PR3)

Question 10

Granulomatosis w/ polyangiitis other name

Answer 10

Wegeners

Question 11

Granulomatosis w/ polyangiitis signs

Answer 11

Upper RT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, saddle nose
Lower RT: hemoptysis, cough, dyspnea
Kidneys: hematuria, red cell casts

Question 12

Granulomatosis w/ polyangiitis histology

Answer 12

Necrotizing granulomas of small vessels, necrotizing glomerulonephritis, necrotizing granulomas in lung/upper airway

Question 13

Polyarteritis nodosa demographics

Answer 13

Young/middle aged men

Question 14

Polyarteritis nodosa association

Answer 14

Hep B pos in 30%

Question 15

Polyarteritis nodosa signs

Answer 15

Fever, wt. loss, malaise, headache
GI: abdominal pain, melena
HTN, neuro dysfxn, cutaneous eruptions, renal damage
NO LUNGS

Question 16

Polyarteritis nodosa histology

Answer 16

Fibrinoid necrosis and thrombosis alternating w/ regions of vascular wall fibrosis

Question 17

Kawasaki disease demographics

Answer 17

Asian child <4yrs

Question 18

Kawasaki other name

Answer 18

Mucocutaneous lymph node syndrome

Question 19

Kawasaki signs

Answer 19

Conjunctival injxn, rash (polymorphous–>desquamating), Adenopathy (cervical), Strawberry tongue, Hand/foot changes (edema, erythema), fever

CRASH and burn

Question 20

Kawasaki complications

Answer 20

Coronary artery aneurysms, thrombosis, rupture –> death

Question 21

Tx of Kawasaki

Answer 21

IVIG and aspirin

Question 22

Microscopic polyangiitis signs

Answer 22

Involvement of kdiensy, lung, skin -- pauci-immune glomerulonephritis and palpable purpura
NO NASOPHARYNGEAL (like in GwP)

Question 23

Microscopic polyangiitis histology

Answer 23

Similar to polyarteritis nodosa w. fibrinoid necrosis except w/ smaller vessels, fragmented neuts (leukocytoclasia), and similar age of lesions

Question 24

Henoch schonlein purpura demographic

Answer 24

Most common systemic vasculitis in kiddos

Question 25

Henoch schonlein purpura association

Answer 25

Follow URI, IgA nephropathy (Berger disease)

Question 26

Triad of Henoch Schonlein Purpura

Answer 26

Skin -- palpable purpura Arthralgias GI: abdominal pain

Question 27

Henoch Schonlein purpura histology

Answer 27

Necrotizing vasculitis w/ depositions of IgA immune complexes and C3 (also in glomerular mesangium)

Question 28

Thromboangiitis obliterans other name

Answer 28

Buerger disease

Question 29

Thromboangiitis obliterans demographic

Answer 29

heavy smoking men under 40

Question 30

Thromboangiitis obliterans signs

Answer 30

Intermittent claudication-->gangrene/autoamputation of digits

Question 31

Thromboangiitis obliterans histology

Answer 31

Segmental thrombosing vasculitis, fibrosis, extension into veins/nerves --> painful

Question 32

Eosinophilic granulomatosis w/ polyangiitis other name

Answer 32

Churg Strauss

Question 33

Eosinophilic granulomatosis w/ polyangiitis histology

Answer 33

Lots and lots of eo's w/ granulomas

Question 34

Eosinophilic granulomatosis w/ polyangiitis signs

Answer 34

Asthma, sinusitis, skin nodules, purpura, peripheral neuropathy (foot/wrist drop), heart, GI, kidneys (pauci-immune glomerulonephritis)

Question 35

Eosinophilic granulomatosis w/ polyangiitis demographic

Answer 35

Middle aged w/ long history of asthma

Question 36

Eosinophilic granulomatosis w/ polyangiitis association

Answer 36

p-ANCA (anti-MPO), increaed IgE

Question 37

Hereditary hemorrhagic telangietasia findings

Answer 37

Blanching skin lesions, recurrent epistaxis, skin discolorations, AVMs, GI bleeding, hematuria

Question 38

Hereditary hemorrhagic telangiectasia other name

Answer 38

Osler Weber Rendu