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GIANT CELL
Vessel Size
Smallmedium Arteries
AND
veins1
Arteritis Chart Pathology Segmental acute & chronic inflammation of arterial wall. Thrombosis of lumen w. giant cell granuloma formation. (organization recanalization) No necrosis.
Large
Temporal
Older
Mediumlarge
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
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
Small
SLE Arteritis
Non-GIANT CELL
Small
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
Vessel Size
Location systemic
Symptoms
side of mitral valve
Diagnosis
small
Non-GIANT CELL
Raynauds2
Small
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
Comorbid infection.
Variable
HenochSchnlein Purpura
Kawasakis Disease
Young kids, winter, preceded by viral or GrpA Strep URI Infants & young kids >
Small
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
1
2
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.