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35 Cards in this Set
- Front
- Back
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What are ANCA's?
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Antineutrophil Cytoplasmic Antibodies (serum antibodies to neutrophils)
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What are the two types of ANCA's and what do they bind?
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c-ANCAs bind proteinase 3 (Pr-3) located within the cytoplasm
p-ANCAs bind myeloperoxidase (MPO) located in teh perinuclear area |
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What is the hypothetic mechanism of action of ANCAs?
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1. ANCAs bind to neutrophils with expressed MPO and PR-3 on their cell surface
2. Neutrophils accumulate in microvasculature 3. Neutrophils degranulate 4. Neutrophil granular content injures vessel wall and induces inflammatory reaction |
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What is the epidemiology of Wegener Granulomatosis?
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NECROTIZING GRANULOMATOUS VASCULITIS
Adults : any age, ave 50yrs M≥W C-ANCA (PR-3) |
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What are three key features of Wegener Granulomatosis?
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1. Granulomatous vasculitis of small vessels
2. Upper airway (especially middle) or lung necrotizing granulomas 3. Kidney lesions (necrotizing glomerulonephritis) |
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What are clinical manifestations of Wegener Granulomatosis?
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- Recurrent pneumonia
- Chronic sinusitis - Nasopharyngeal ulcers +/- SADDLE NOSE DEFORMITY -Kidney damage (nephritic syndrome) *Tx with immunosuppression : fatal if left untreated |
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Large Vessel Vasculitides
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*Granulomatous
- Giant Cell (Temporal) Vasculitis - Takayasu Arteritis |
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Medium Vessel Vasculitides
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*Anti-Endothelial or Immune Complex
- Polyarteritis nodosa -Kawasaki Disease |
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Small Vessel Vasculitides
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*ANCA or immune complex
- Microscopic polyangiitis - Churg-Strauss Syndrome -Wegener granulomatosis |
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What is the epidemiology of Microscopic Polyangiitis? (p-ANCA {MPO})
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Age ~50yrs
M≥F Whites>Blacks *Thought to be hypersensitivity reaction to drugs, toxins, microbes, etc. {Aspirin, penicillin, thiazide diuretics} |
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What is the histological feature of Microscopic Polyangiitis?
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SEGMENTAL NECROTIZING VASCULITIS
-vasculitis without asthma or granulomas - Polymorpho / mononuclear infiltration - Fibrinoid necrosis |
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What are the clinical features of Microscopic Polyangiitis?
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- SKIN: palpable purpuric rash, lower extremities
- KIDNEYS: Glomerulonephritis (Nephritic Syndrome) - RESPIRATORY TRACT: Sinusitis, pneumonitis, cough, hemootysis - GI Tract: +/- bleeding *Tx by eliminating causative agent *Good prognosis except in lung or kidney involvement |
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What is the epidemiology of Churg-Strauss Syndrome? (p-ANCA {MPO})
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Adults: Ave age 50yrs
M≥F Very rare (1:1,000,000) *AKA: allergic granulomatosis and angiitis |
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What is the histological feature of Churg-Strauss Syndrome?
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NECROTIZING GRANULOMATOUS VASCULITIS WITH EOSINOPHILS
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What are the clinical features of Churg-Strauss Syndrome?
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*BRONCHIAL ASTHMA
* ALLERGIC PARANASAL SINUSITIS * EOSINOPHILIA - Also, Transient pulmonary infiltrates and purpuric skin rash |
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What is the epidemiology of Polyarteritis Nodosa? (PAN)
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Young Adults
M≥F 30% Patients have HEPATITIS B ANTIBODIES |
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What is the histological feature of Polyarteritis Nodosa?
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SEGMENTAL TRANSMURAL NECROTIZING ARTERITIS of medium-sized and small MUSCULAR arteries
-sharply segmental, transmural -mixed inflammantion - fibrinoid necrosis -nodular fibrosis (older lesions) * all stages of inflammation and healing may coexist in the same or different vessels |
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Where does Polyarteritis Nodosa affect?
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-KIDNEYS, GI Tract, Skin
- Causes nodular thickening of the vascular wall - +/- aneurysmal dilation (<0.5cm) with hemorrhage, thrombosis and infarctions in the supplied areas *Pulmonary Vasculature not affected. |
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What are the clinical features of Polyarteritis Nodosa?
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- KIDNEY: nephritic syndrome, renal failure
- GI Tract: abdominal pain and melena (black feces bc hemorrhage) - Musculoskeletal: diffuse muscular aches and pains - Integument: painful skin, SUBCUTANEOUS NODULES -Systemic: Fever, Malaise, Weight Loss *Tx with immunosuppression *Fatal if left untreated |
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What is the epidemiology of Kawasaki Disease?
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Infants and Young Children
- Leading cause of acquired heart diseases of children in North America and Japan -Induced by viral or bacterial superantigens - Anti-endothelial antibodies -AKA: Mucocutaneous lymph node syndrome |
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What is the histological feature of Kawasaki Disease?
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TRANSMURAL NECROTIZING ARTERITIS
- transmural inflammation - progresses to fibrosis and stenosis |
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Where does Kawasaki disease affect?
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*CORONARY ARTERIES (70% all cases)
* skin, mucous membrane, lymph nodes *Can lead to CORONARY ARTERY ANEURYSM |
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What are the clinical manifestations of Kawasaki Disease?
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*SKIN RASH, lymphadenopathy, fever, malaise
*Conjunctival and ORAL EROSIONS / ERYTHEMA *Cardiac Manifestations Tx: aspirin and IV gamma-globulin *Disease is usually self limited but cardiac involvement may be fatal due to ACUTE MI OR RUPTURE OF CORONARY ARTERY ANEURYSM |
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What is the epidemiology of Giant Cell (Temporal) Arteritis?
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Most common vasculitis in adults
F≥ M ~50yrs of age *Cell mediated immune reaction (elastin antigen??) |
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What is the histological feature of Giant Cell Arteritis?
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SEGMENTAL GRANULOMATOUS INFLAMMATION
- media and intima - MULTINUCLEATED GIANT CELLS - necrosis and fragmentation of the internal elastic membrane - Later stage: intimal thickening and medial fibrosis |
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What arteries are affected in Giant Cell Arteritis and what is their gross appearance?
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Cranial arteries
- SUPERFICIAL TEMPORAL -Ophthalmic -Facial Gross appearance: -Segmental nodular thickening - Lumen narrowing +/- Thrombosis |
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What are the clinical manifestations of Giant Cell Arteritis?
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- Superficial temporal artery is thickend, swollen and tender
- Unilateral HEADACHE -Ipsilateral visual loss - Polymyalgia rheumatica (pain and morning stiffness in the neck, shoulders and hips) - Fever, malaise |
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How is Giant Cell Arteritis Diagnosed and Treated?
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Dx: Temporal Artery Biopsy
Tx: Anti-inflammatory drugs (corticosteroids) Prognosis is good: subsides in 6-12 months. Blindness if left untreated |
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What is the epidemiology of Takayasu Arteritis?
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F>M (F ~90%)
>40 (30) yrs of age - Asians Immune mechanism?? AKA: Pulseless Disease |
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What is the histological feature of Takayasu Arteritis?
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GRANULOMATOUS INFLAMMATION WITH INTIMAL FIBROSIS
- GIANT CELL GRANULOMAS - Transmural mononuclear inflammation - Later changes: Fibrosis and intimal proliferation |
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What arteries are affected in Takayasu Arteritis and what is their appearance?
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* NARROWING OF AORTIC ARCH AND MAJOR BRANCHES (Subclavian, Carotid)
- Irregular thickening (intimal fibrosis) of the vascular wall - Intimal wrinkling |
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What are the clinical manifestations of Takaysu Arteritis?
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* Decreased blood flow through carotids and subclavians (pulseless disease)
Tx: Corticosteroids *Prognosis is usually good but is worse with stroke or aortic dissection |
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Henoch - Schönlein Purpura
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* IgA ASSOCIATED VASCULITIS
* Most common Vasculitis in kids * Follows infection (Viral, Strep A) *IgA & COMPLEMENT IMMUNE DEPOSITS *Involves skin, GI, Renal, Joints *Self - Limited |
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Raynaud Disease
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*COLD INDUCED VASOSPASM
* Fingers and toes * Part of CREST syndrome * Not really a Vasculitis |
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Thromboangiitis Obliterans (Buerger Disease)
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* SMOKERS
* Vasculitis with thrombosis * Involves extremities (Gangrene) * Tx = stop smoking |
