Vasculitis Flashcards
1
Q
Vasculitis Classification by Vessel Size
- Large vessel
- Medium vessel
- Small vessel
A
- Large vessel
- Giantcell (temporal) vasculitis
- Takayasu arteritis
- Immunology: granulomatous
- Medium vessel
- Polyarteritis nodosa
- Kawasaki disease
- Immunology: anti-endothelial or immune complex
- Small vessel
- Microscopic polyangiitis
- Churg-Strauss syndrome
- Wegener granulomatosis
- Immunology: antineutrophil cytoplasmic antibodies (**ANCA) **or immune complex
2
Q
Anti-Neutrophil Cytoplasmic Antibodies (ANCA)
- 2 types
- Serum antibodies to neutrophils
- Hypothetic mechanism of action
A
- 2 types
- c-ANCA (PR-3) cytoplasmic
- p-ANCA (MPO) perinuclear
- Serum antibodies to neutrophils
- c-ANCAs bind proteinase 3 (PR-3) located within the Cytoplasm
- p-Ancas bind myeloperoxidase (MPO) located at the Perinuclear area
- Hypothetic mechanism of action
- ANCAs bind to neutrophils w/ expressed MPO & PR-3 on their cell surface
- Neutrophils accumulate in microvasculature
- Neutrophils degranulate
- Neutrophil granular content injures vessel wall & induces inflammatoyr reaction
3
Q
Wegener Granulomatosis
- Size
- ANCA type
- General
- Key features
- Clinical manifestations
- Treatment
- Summary
A
- Size
- Small vessel
- ANCA type
- c-ANCA PR3
- General
- Necrotizing granulomatous vasculitis
- Adults: any age, avg = 50yo
- M > F
- Key features
- Granulomatous vasculitis of small vessels
- Upper airway or lung necrotizing granulomas
- Kidney lesions (necrotizing glomerulonephritis
- Clinical manifestations
- Recurrent pneumonia
- Chronic sinusitis
- Nasopharyngeal ulcers +/- saddle nose deformity
- Kidney damage (nephritic syndrome)
- Treatment
- Immunosuppression
- Fatal if untreated
- Summary
- Granulomatous vasculitis of small vessels
- No asthma
- Upper airway, lung, & kidney lesions (esp midline upper airway)
- c-ANCA (PR3)
4
Q
Microscopic Polyangiitis
- Size
- aka
- ANCA type
- General
- Primarily affects…
- Clinical manifestations
- Treatment
- Prognosis
- Summary
A
- Size
- Small vessel
- aka
- Hypersensitivity vasculitis
- Leukocytoclastic vasculitis
- Microscopic polyarteritis
- ANCA type
- p-ANCA (MPO)
- General
-
Segmental necrotizing vasculitis
- Polymorphonuclear / mononuclear infiltration
- Fibrinoid necrosis
- Kidneys: necrotizing & crescentic glomerulonephritis
- No granulomas & no asthma
- Age: ~50yo
- M > F
- Whites > blacks
-
Hypersensitivity reaction to drugs or toxins
- Antigens: drugs, aspirin, penicillin, thiazide diuretics, microorganisms, etc.
-
Segmental necrotizing vasculitis
- Primarily affects…
- Skin (purpura = rash)
- Kidneys (glomerulonephritis)
- Respiratory tract (sinusitis, pneumonitis)
- GI tract
- Clinical manifestations
- Palpable purpuric rash, lower extremities
- Nephritic syndrome (kidney damage)
- Sinusitis, cough, hemoptysis
- +/- GI hemorrhage
- Treatment
- Eliminating causative agent
- Prognosis
- Good except in kidney &/or lung involvement
- Summary
- Segmental necrotizing vasculitis w/ no granluomas & no asthma
- Hypersensitivity reaction to drugs, toxins, microbes, etc.
- p-ANCA (MPO)
5
Q
Churg-Strauss Syndrome
- aka
- ANCA type
- General
- Primarily affects…
- Clinical manifestations
- Prognosis
- Summary
A
- Size
- Small vessel
- aka
- Allergic granulomatosis
- Angiitis
- ANCA type
- p-ANCA (MPO)
- General
- Necrotizing granulomatous vasculitis w/ eosinophils
- Adults: mean age = 50yo
- M > F
- Very rare
- Bronchial asthma, allergic paranasal sinusitis, & eosinophilia
- Primarily affects…
- Lungs
- Paranasal sinuses
- Skin
- Heart
- Peripheral nerves
- Clinical manifestations
- Asthma
- Transient pulmonary infiltrates
- Purpuric skin rash
- Prognosis
- 60% 5-year survival rate
- Summary
- Vasculitis w/ asthma & granulomas
- p-ANCA (MPO
6
Q
Small Vessel Vasculitis Summary
- Wegener
- Granulomas?
- ANCA type
- Other
- Microscopic polyangiitis
- Granulomas?
- ANCA type
- Other
- Churg-Strauss
- Granulomas?
- ANCA type
- Other
A
- Wegener
- Granulomas
- c-ANCA (PR3)
- Nose, lung, kidney
- c-ANCA (PR3)
- Microscopic polyangiitis
- No granulomas
- p-ANCA (MPO)
- Hypersensitivity drug reaction
- Churg-Strauss
- Granulomas
- p-ANCA (MPO)
- Eosinophils, asthma
7
Q
What is the treatment for microscopic polyangiitis?
A
Stpo taking the offending drug
8
Q
Polyarteritis Nodosa (PAN)
- Size
- General
- Primarily involves…
- Gross appearance
- Histology
- Manifestations
- Treatment
- Summary
A
- Size
- Medium vessel
- General
- Segmental transmural necrotizing arteritis
-
Medium-sidzed & small musclar arteries
- Unlike microscopic polyangiitis that affects arterioles, capillaries, & venules
- Immune complexes (no ANCA association)
- 30% of patients have Hepatitis B antibodies
- Young adults
- M > F
- Primarily involves…
- Kidneys
- GI tract
- Skin
- Pulonary vasculature isn’t affected
- Gross appearance
- Nodular thickening of the vascular wall
- +/- aneurysmal dilation (<0.5 cm) w/ hemorrhage, thrombosis, & infarctions in the supplied areas
- Histology
- Sharply segmental, transmural
- Mixed inflammation
- Fibrinoid necrosis
- Nodular fibrosis (older lesions)
- All stages of inflammation & healing may coexist in the same or different vessels
- Manifestations
- Kidney: nephritic syndrome, renal failure
- GI tract: abdominal pain, melena
- Musculoskeletal: diffuse muscular aches & pains
- Integument: painful skin, subcutaneous nodules
- Systemic: fever, malaise, weight loss
- Treatment
- Immunosuppression
- Fatal if untreated
- Summary
- Segmental necrotizing vasculitis
- Hepatitis B antibodies in 30% of cases
- Young adults
- Renal failure: cause of 2o hypertension
9
Q
Kawasaki Disease
- Size
- aka
- General
- Primarily involves…
- Locaiton
- Clinical manifestations
- Gross appearance
- Histology
- Treatment
- Summary
A
- Size
- Medium vessel
- aka
- Mucocutaneous lymph node syndrome
- General
- Transmural necrotizing arteritis
- Infants & young children (<5yo)
- Leading cause of acquired heart diseases of children in North America & Japan
- Induced by viral or bacterial superantigens
- Anti-endothelial cell antibodies
- Primarily involves…
- Coronary arteries
- Skin
- Mucous membranes
- Lymph nodes
- Location
- Arteries of any caliber
- Esp coronary arteries (70% of all cases)
- Clinical manifestations
- Skin rash, lymphadenopathy, fever, malaise
- Conjunctival & oral erosions / erythema
- Cardiac manifestatoins (20%)
- Gross appearance
- Severe cases have coronary artery aneurysm
- Histology
- Transmural inflammation
- Progresses to fibrosis & stenosis
- Treatment
- IV gamma-globulin
- Disease is usually self-limited
- Cardiac involvement w/o treatment may be fatal due to acute MI or rupture of coronray artery aneurysm
- Summary
- Young children
- Coronary artery involvement (aneurysm)
- Viral or bacterial infection
- Rash & “strawberry tongue”
10
Q
Medium Vessel Vasculitis Summary
- Polyarteritis nodosa
- Kawasaki disease
A
- Polyarteritis nodosa
- Young adults
- Segmental necrotizing vasculitis
- Hepatitis B antibodies (30% of cases)
- Renal failure
- Kawasaki disease
- Young children
- Coronary artery involvement (aneurysm)
- Viral or bacterial infection
- Rash & “strawberry tongue”
11
Q
Giant Cell (Temporal) Arteritis
- Size
- General
- Mechanism
- Clinical manifestations
- Gross appearance
- Histology
- Diagnosed by…
- Treatment
- Prognosis
- Summary
A
- Size
- Large vessel
- General
- Segmental granulomatous inflammation
- Cranial arteries
- Superficial temporal artery
- Ophthalmic artery
- Facial artery
- Most common vasculitis in adults
- F > M
- Age = 50yo
- Mechanism
- Cell-mediated immune reaction
- Antigen: elastin?
- Clinical manifestations
- Superficial temporal artery is thickened, swollen, & tender
- Unilaterla headache
- Ipsilateral visual loss
- Polymyalgia rheumatica: pain & morning stiffness in the neck, shoulders, & hips
- Fever, malaise
- Gross appearance
- Segmental nodular thickening
- Lumen narrowing +/- thrombosis
- Histology
- Granulomatous inflammation
- Media & intima
- Multinucleated giant cells
- Necrosis & fragmentation of the internal elastic membrane
- Later stage: intimal thickening & medial fibrosis
- Diagnosed by…
- Termporal artery biopsy
- Treatment
- Anti-inflammatory drugs (corticosteroids)
- Prognosis
- Good, subsides in 6-12 months
- Blindness if untreated
- Summary
- Granulomatous inflammation
- Temporal artery
- Females
12
Q
Takayasu Arteritis
- Size
- aka
- General
- Clinical manifestations
- Mechanism
- Gross appearance
- Microscopic appearance
- Treatment
- Prognosis
- Summary
A
- Size
- Large vessel
- aka
- Giant-cell aortitis
- General
- Granulomatous inflammaitonw/ massive intimal fibrosis
- Aortic arch & its major branches
- F > M (F ~ 90%)
- Two age ranges
- <50yo = Takayasu arteritis
- >50yo = Giant-cell aortitis
- Clinical manifestations
- Related to decreased blood flow through carotids & subclavians (“pulseless diseease”)
- Initially nonspecific (fever, fatigue)
- Ocular changes (visual defects, blindness)
- Neurologic defects
- Coronary arteries –> myocardial infarcts
- Pulmonary artery –> pulmonary hypertension
- Mechanism
- Immune?
- Japanese populations w/ HLA subtype
- Gross appearance
- Irregular thickening (intimal fibrosis) of the vascular wall
- Intimal wrinkling
- Narrowing of the orifices of major arterial branches (carotid, subclavian)
- Involves pulmonary artery in 50% of cases
- Microscopic apperance
- Giant cell granulomas
- Transmural mononuclear inflammatoin
- Later changes: fibrosis & intimal proliferation
- Treatment
- Corticosteroids
- Prognosis
- Usually good
- Worse w/ stroke or aortic dissection
- Summary
- Granulomatous inflammation
- Carotid, subclavian (aortic arch)
- “Pulseless disease”
13
Q
Which of the following vasculitides has an ANCA association?
- Churg-Strauss syndrome
- Polyarteritis nodosa
- Takayasu arteritis
- Giant cell temporal arteritis
- Kawasaki disease
A
Churg-Strauss syndrome
14
Q
Thromboaniitis Obliterans (Buerger Disease)
A
- Smokers
- Vasculitis w/ thrombosis
- Involves extremities (gangreen)
- Treatment: stop smoking
15
Q
Henoch-Schönlein Purpura
A
- IgA associated vasculitis
- Most common vasculitis in children
- Follows infection (viral, Strep A)
- IgA & complemetn immune deposits
- Involves skin, GI, renal, joints
- Self-limited
