Vasculitis Flashcards Preview

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Flashcards in Vasculitis Deck (23):
1

Vasculitis Classification by Vessel Size

  • Large vessel
  • Medium vessel
  • Small vessel

  • 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

Anti-Neutrophil Cytoplasmic Antibodies (ANCA)

  • 2 types
  • Serum antibodies to neutrophils
  • Hypothetic mechanism of action

  • 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

Wegener Granulomatosis

  • Size
  • ANCA type
  • General
  • Key features
  • Clinical manifestations
  • Treatment
  • Summary

  • Size
    • Small vessel
  • ANCA type
    • c-ANCA PR3
  • General
    • Necrotizing granulomatous vasculitis
    • Adults: any age, avg = 50yo
    • > 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

Microscopic Polyangiitis

  • Size
  • aka
  • ANCA type
  • General
  • Primarily affects...
  • Clinical manifestations
  • Treatment
  • Prognosis
  • Summary

  • 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
    • > F
    • Whites > blacks
    • Hypersensitivity reaction to drugs or toxins
      • Antigens: drugs, aspirin, penicillin, thiazide diuretics, microorganisms, etc.
  • 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

Churg-Strauss Syndrome

  • aka
  • ANCA type
  • General
  • Primarily affects...
  • Clinical manifestations
  • Prognosis
  • Summary

  • Size
    • Small vessel
  • aka
    • Allergic granulomatosis
    • Angiitis
  • ANCA type
    • p-ANCA (MPO)
  • General
    • Necrotizing granulomatous vasculitis w/ eosinophils
    • Adults: mean age = 50yo
    • > 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

Small Vessel Vasculitis Summary

  • Wegener
    • Granulomas?
    • ANCA type
    • Other
  • Microscopic polyangiitis
    • Granulomas?
    • ANCA type
    • Other
  • Churg-Strauss
    • Granulomas?
    • ANCA type
    • Other

  • 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

What is the treatment for microscopic polyangiitis?

Stpo taking the offending drug

8

Polyarteritis Nodosa (PAN)

  • Size
  • General
  • Primarily involves...
  • Gross appearance
  • Histology
  • Manifestations
  • Treatment
  • Summary

  • 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
    • > 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

Kawasaki Disease

  • Size
  • aka
  • General
  • Primarily involves...
  • Locaiton
  • Clinical manifestations
  • Gross appearance
  • Histology
  • Treatment
  • Summary

  • 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

Medium Vessel Vasculitis Summary

  • Polyarteritis nodosa
  • Kawasaki disease

  • 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

Giant Cell (Temporal) Arteritis

  • Size
  • General
  • Mechanism
  • Clinical manifestations
  • Gross appearance
  • Histology
  • Diagnosed by...
  • Treatment
  • Prognosis
  • Summary

  • Size
    • Large vessel
  • General
    • Segmental granulomatous inflammation
    • Cranial arteries
      • Superficial temporal artery
      • Ophthalmic artery
      • Facial artery
    • Most common vasculitis in adults
    • > 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

Takayasu Arteritis

  • Size
  • aka
  • General
  • Clinical manifestations
  • Mechanism
  • Gross appearance
  • Microscopic appearance
  • Treatment
  • Prognosis
  • Summary

  • 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

Which of the following vasculitides has an ANCA association?

  • Churg-Strauss syndrome
  • Polyarteritis nodosa
  • Takayasu arteritis
  • Giant cell temporal arteritis
  • Kawasaki disease

Churg-Strauss syndrome

14

Thromboaniitis Obliterans (Buerger Disease)

  • Smokers
  • Vasculitis w/ thrombosis
  • Involves extremities (gangreen)
  • Treatment: stop smoking

15

Henoch-Schönlein Purpura

  • IgA associated vasculitis
  • Most common vasculitis in children
  • Follows infection (viral, Strep A)
  • IgA & complemetn immune deposits
  • Involves skin, GI, renal, joints
  • Self-limited

16

Raynaud Disease / Phenomenon

  • Cold-induced vasospasm
  • Involves digits of hands & feet
  • Part of CREST syndrome or other disease (Raynaud phenomenon)
  • Not really a "vasculitis"

17

Scenario 1

  • 50yo man
  • Recurrent sinusitis, pneumonia, & nasal ulcers
  • Now has renal failure
  • Kidney biopsy shows small vessel granulomatous vasculitis & glomerulonephritis

Wegener's Granulomatosis

18

Scenario 2

  • 45yo man
  • Just started taking a new antibiotic for sinusitis
  • Develops a skin rash & shows signs of renal failure
  • Kidney biopsy shows a small vessel necrotizing vasculitis w/o granulomas
  • He quits taking the antibiotic & his symptoms improve

Microscopic Polyangiitis (hypersensitivity vasculitis)

19

Scenario 3

  • 56yo man
  • Asthma since childhood
  • Develops a skin rash
  • Chest x-ray shows patchy pulmonary infiltrates
  • Lung biopsy shows small vessel necrotizing vasculitis w/ granulomas & eosinophils

Churg-Strauss Syndrome

20

Scenario 4

  • 24yo woman
  • Hepatits B
  • Develops renal failure
  • Ultrasound: multiple renal infarcts
  • Kidney biopsy: medium vessel vasculitis
    • Sharply segmental
    • Mixed ifnlammation
    • Focal fibrinoid necrosis

Polyarteritis Nodosa

21

Scenario 5

  • 3yo male child
  • Recovering from an apparent viral infection
  • Develops a rash w/ oral & conjunctival erythema, cervical lymphadenopathy, & is lethargic
  • What are you most worried about?

Coronary artery involvement

Mucocutaneous lymph node syndrome

Kawasaki disease

22

Scenario 6

  • 51yo woman
  • Left-sided headaches
  • Slight vision loss in the left eye
  • Bulging, palpable left temporal artery that on biopsy has granulomatous inflammation

Giatn Cell Temporal Arteritis

23

Scenario 7

  • 35yo woman
  • Japanese ancestry
  • Weakened upper extremity pluses
  • Progressive visual loss
  • Aortography: diffuse narrowing of aortic arch, brachiocephalic, subclavian, & carotid arteries

Takayasu Arteritis

If over 50yo: Giant Cell Aortitis