Vascular/Autoimmune Conditions

Question 1

Temporal Giant Cell Arteritis:

Etiology, Presentation, Pathology, Labs, Tx?

Answer 1

50+ Females, Headache (Temporal A), Visual (Opthalmic A), Jaw Claudication.

Associated with Polymyalgia Rheumatic: mild synovitis in Joint/hips; flu-like sxs w/ elevated ESR.

Granulomatous vasculitis between the inima and media (causing narrowed lumen) with Giant Cells and intimal fibrosis. “disrupted internal elastic lamella”

Usually presents with cranial vessel invovlement but usually invovles subclavian and aortic too (dx via PET)

Tx: Med emergency get Steroids ASAP to PREVENT VISION LOSS

Question 2

Takayasu Arteritis

Etiology, Presentation, Pathology, Labs, Tx?

Answer 2

Asian Females <40 yo affecting arteries closer to heart: AORTIC ARCH AND PRIMARY BRANCHES (compared with temporal giant cell which is arteries more in head)

“Pulseless disease” (weak upper extremity pulses) with Fever, night sweats, arthtisis, myalsias,.

Claudication in young female (Takaysua until proven otherwise)

Granulomatous vasculitis between the inima and media (causing narrowed lumen) with Giant Cells and intimal fibrosis

Tx: Corticosteroids

Question 3

Polyarteritis Nodosa

Etiology, Presentation, Pathology, Labs, Tx?

Answer 3

Young Adults (Hep B in 30%). String of Pearls

Renal and Visceral Vessel involvement leading to HTN and GI pain/melena.

Type 3 HSR leading to transmural inflammation of wall with fibrinoid necrosis.

Tx: Corticosteroids and Cyclophosphamide

Question 4

Kawasaki Disease

Etiology, Presentation, Pathology, Labs, Tx?

Answer 4

Young asain kid (less than 4) on Kawasaki motorcyles with rash on hands and feet and having MI (Beefy lips and strawberry tongue)

Present with very nonspecific viral infectious type signs
HIGH RISK FOR CORONARY ARTERY inovlement=MI; or risk for aneurisyms progressing to rupture

TX: GIVE ASPIRIN************** and IVIG:
Only time you give kids aspirin (risk fo reye’s syndrome); aspirin knocks down TXA2 protecting against thrombus formation

Disease is self limitied

Question 5

Buerger Disease (thromboangiitis obliterans).

Etiology, Presentation, Pathology, Labs, Tx?

Answer 5

“Segmental Vasculitis extending into contigous veins and nerves”

Heavy male smoker <40yo

Necrotizing vascultis involving the digits

Ulceration gangrene and autoamputation of fingers/toes, Raynaud phenomen*****

ASSOCIATED WITH SMOKING=thus stop smoking

“bascially HSR to a tobacco product”

Question 6

Granulomatosis with polyangiitis (Wegener’s)

Etiology, Presentation, Pathology, Labs, Tx?

Answer 6

Wegener Granulomatosis “weCners”—C disease

1. C shaped involvement of organs=nasopharynx, lungs, and kidneys (upper resp tract, lower resp tract, kidneys))
2. C-ANCA (levels correlate with disease activity)
3. Cyclophosphmaide (Common for relapses)

Middle aged male, sinusitis, nasopharyngeal ulceration, hemoptysis, bilateral nodular lung infiltrates, hematuria due to RPGN******

Large necrotizing granulomas with adjacent necrotizing vasculitis

Question 7

Microscopic Polyangiitis

Etiology, Presentation, Pathology, Labs, Tx?

Answer 7

Necrotizing vasculitis involving multiple organs (LUNG AND KIDNEYS)

Different from Wegneers by:

i. NO NASOPHARYNGEAL INVOLVEMENT
ii. NO GRANULOMATOMAS (churg and wegeners do)
iii. P-ANCA (MPO)

Tx: Corticosteroids and Cyclophosphamide

Question 8

Churg Strauss Syndrome (EGPA)

Etiology, Presentation, Pathology, Labs, Tx?

Answer 8

Eosinophillic Granulomatous Polyangitis

Necrotizing granulomatous vasculitis with EOSINOPHILS (elevated IgE too)***

Involves multiple organs (especially lungs and heart)

P-ANCA (MPO)

dd with microscopic polyangitis:

i. GRANULOMAS
ii. ASTHMA

iii.PERIPHERAL EOSINOPHILIA

Question 9

Henoch-Schonlein Purpura

Etiology, Presentation, Pathology, Labs, Tx?

Answer 9

URI causing IgA which then deposits in vesseles (notably kidneys) driving vasculitis

Most common vasculitis in kids

Palpable******* purpura (palabable from vasculitis) on buttock and legs, collicy pain from intussception

GI pain and bleeding
Hematuria (IgA nephropathy)—within the glomerul

Usually follows an URI (why IgA)
Treat severe cases with steroids

Question 10

Cryoglobulinemia.

Associated with?

Bugs known to cause it?

Causes:

Answer 10

Protein that “precipates” out during colder temps.

Associated with Hep C (~30%).

Mycoplasma Pneumoniae, Mononucleosis, and HIV

Purpura, Necorsis, glomerular deposits with Eosinophils, Peripheral nerves

Question 11

Behcet Syndrome

Answer 11

HLA-B51, PAINFUL mucosal and genital lesions with Uveitis and Hypopyon (inflammation in anterior chamber of eye)

Variable vessel vasculiditis;

Found along silk road

Question 12

Drug Induced Lupus: Postive for?

Drugs?

Answer 12

Antihistone Antibodies

“SHIPP”

Sulfonamides, Hydralazine, Isoniazid, Phenytoin, Procainamide (and Methyldopa)

Question 13

Granulomatous Inflammation of the media?

Answer 13

Temporal Giant Cell Arteritis

Question 14

Transmural Inflammation with Fibrinoid Necrosis

Answer 14

Polyarteriitis Nodosa

Can also seen in malignant HTN, preeclampsia

Question 15

Transmural Infarct seen in?

Answer 15

Polyarteritis nodosa

Question 16

Polyarteritis nodosa does not affect?

Answer 16

The lungs

Question 17

Intimal Injury of vessels?

Answer 17

Chemicals/chronic inflammation causes damaged endothelial cells to secrete growth factors like (PDGF and FGF) that recruit SMOOTH MUSCLE CELLS (not fibroblasts). Reactive intimal hyperplasia results with smooth muscle cell synthesis of collagen, proteoglycans and elastin.

Question 18

Amlodipine:

MOA, SEs?

Answer 18

Dihydropyradine Calcium channel blocker decreasing peripheral resistance.

SEs: PERIPHERAL EDEMA due to above (and flushing)