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Cardiovascular Domain Arteritis Epid Thromboangitis Young Obliterans smokers (<35y) Buergers Dz >

GIANT CELL

Vessel Size

Smallmedium Arteries
AND

Location Arms & legs (tibial&radial arteries)

veins1

Arteritis Chart Pathology Segmental acute & chronic inflammation of arterial wall. Thrombosis of lumen w. giant cell granuloma formation. (organization recanalization) No necrosis.

Takaysaus Arteritis Pulseless dz


GIANT CELL

Young (<40y) Asia, Africa

Large

Aortic arch 1/3 of cases involve the desc. aorta

Temporal

Older

Mediumlarge

Extra-cranial branches of carotid

GIANT CELL

Gross: marked fibrosis w. thickening of aortic arch w. narrowing/ obliteration of ostia of vessels branching from arch. Micro: Intimal hyperplasia, medial & adventitial thickening (panarteritis = all 3 layers). Giant cell granuloma formation. Gross: Intimal fibrosis w. thickening in short arterial segments lumen size. Micro: Granuloma formation w. multinucleated giant cells Necrotizing inflammation d/t immune complex formation in small-med muscular arteries of viscera (esp kidney) microaneurysms Focal necrotizing inflammation w. giant cell granuloma formation

Symptoms Distal arterial occlusion, Raynauds phenomenon, migrating superficial vein thrombophlebitis. Foot instep claudication. Ulceration gangrene of fingers & toes. Diminished or absent pulses in upper extremities & carotid. Arm claudication syncope, TIA ocular disturbances fever, nt sweats, myalgia, arthritis Jaw claudication (jaw pain or limpness when chewing) Headaches (temporal a) Facial pain (facial a) Visual impairment (ophthalmic a)
Fever, wt loss, malaise, abdominal pain, HA, myalgia, HTN, palpable purpura, painful erythematous skin nodules, neuropathy

Diagnosis

Stephanie Commings, M.D. Tx Other Stop Etiology: may be smoking!! hypersensitivity rxn to tobacco.

Symptoms + ESR

Steroids Surgical bypass

Bad prognosis: 40% mortality in 10 years

Polyarteritis Nodosa (PAN)

Hep B + (30%)

Smallmedium

Kidneys, coronary, muscles, GI, CNS, eye, skin Lung, upper airways, kidney

Definitive =Temporal artery bx Suspect if temporal a is TTP + ESR P-ANCA ESR vessel biopsy CXR: nodular densities RBC casts +hematuria C-ANCA

NSAIDs Prednisone

Inflammation of ophthalmic artery blindness.


50% have associated polymyalgia rheumatica (PMR) a collagen-vascular dz

Steroids Azathioprine Cyclophosphamide Steroids, cyclophosphamide MTX Prednisone MTX

Mortality d/t renal or cardiac complications (myocarditis, pericarditis)

Wegeners (Pauci Immune)


GIANT CELL

Small

SLE Arteritis

Non-GIANT CELL

9:1 Onset: young adult I: 1/2500 Exacerbated during menses, pregnancy.

Small

Kidneys, heart, systemic

Anti-dsDNA Abs destroy small vessels in Type III rxn necrotizing non-giant cell arteritis. Can be complicated by thrombosis

Perforation of nasal septum, chronic sinusitis, OM, cough, dyspnea, hemoptysis, hematuria, renal insufficiency. Arteritis is variable: subacute chronic.
Other sx: malar rash, glomerulonephritis, arthralgia, fever, blood dyscrasias, Libman-Sachs endocarditis: plt vegetations on ventricular

HLA-DR3 Rx causing SLElike rxn (mediated by anti-histone Abs) -Penicillamine -hydralazine -INH -procainamide

Cardiovascular Domain Arteritis Epid Scleroderma 3 : 1 Onset:50-60y More severe in black


Occurs as PSS (Progressive Systemic Sclerosis) or CREST Syndrome

Vessel Size

Location systemic

Arteritis Chart Pathology Progressive intimal inflammation w. fibrosis + sclerosis.

Symptoms
side of mitral valve

Diagnosis

Stephanie Commings, M.D. Tx Other PSS: AntiTopoisomerase I Abs

small

Non-GIANT CELL

Raynauds2

Young Age 20-40 Seen in SLE, Scleroderma


(PSS & CREST) atherosclerosis,

Small

Fingers, toes, ears, nose

PSS: localized (fingers, forearms, face late onset visceral involvement) OR Diffuse and progressive fibrosis/ sclerosis of kidney, lungs, heart, (pulmonary fibrosis), intenstines, peripheral vasculature death Vasospastic episodes: Triphasic color response: blanch white
cyanosis ruddy w. reperfusion.

CREST: Anticentromere
C: calcinosis:Ca salts in visceral parenchyma R: Raynauds E: esophageal spasm S: sclerodactyly (fingers + toes) T: telangiectasia

Spasm of digital arteries ppt by cold, emotion Proposed etiology:


overactive SNS in vasculatureunopposed vasoconstriction in digits).

Buergers Infectious Arteritis


3

Numbness, pain paresthesias. Variable Variable


Caused by direct invasion of infectious agents, bacterial seeding 2 to septicemia, meningitis, pneumonia, or embolization from endocarditis. Organisms include: TB, syphilis, rickettsia, fungi, viruses, parasites, bacteria.

Comorbid infection.

Variable

Directed at underlying source.

HenochSchnlein Purpura

Kawasakis Disease

Young kids, winter, preceded by viral or GrpA Strep URI Infants & young kids >

Small

GI, skin, joints, kidney

Immunologically mediated vasculitis

Purpuric rash on butt & legs, abdominal pain, vomit, ileus, GI bleed, ptnuria, arthritis
Fever > 5d, dry red cracked lips, strawberry tongue, B/L conjunctivitis, erythema, edema & desquamation of hands & feet, truncal rash, cervical lymphadenopathy

Supportive No steroids

In major vessels (ie. aorta) can lead to weakened wall (mycotic aneurysm) with mycotic embolization. Complicatons d/t glomerulonephritis acute renal failure. Complications: coronary vasculitis & aneurysm, arrhythmias, CHF. Px: depends on cardiac sequelae

Smallmedium

systemic

High dose ASA IVIG Steroids are C/I bc risk aneurysm

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Arteritis = arterial inflammation Phlebitis = venous inflammation Raynauds Disease v. Raynauds Phenomenon/ Syndrome: Raynauds Disease is when the symptoms occur as an isolated disorder in an individual. 3 There are also non-infectious arteritis, such as arteritis due to radiation and chemical toxicity.