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4. HDS Multisystem > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Vasculitis

Definition

A

Inflammation of the blood vessels

Can be classified based on pathology, vessel size or organ involvement

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2
Q

Vasculitis

Epidemiology

A

Most common types:

  • Children: Henoch-Schonlein purpura
  • Adults > 50 y/o: Giant cell arteritis
  • Drug-induced cutaneous vasculitis: unknown prevalence
  • Rare diseases, vary by type
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3
Q

Vasculitis

Pathogenesis

A

Varies w/ vasculitis type

Can have infectious trigger ⇒ pathologic auto-Ab causing immune system activation

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4
Q

Vasculitis

Clinical Presentation

A

Dependent upon type of vasculitis

  • Systemic symptoms:
    • Fever
    • Malaise
    • Weight loss
    • Night sweats
  • Organ-specific manifestations:
    Can affect any organ depending on the type of vasculitis
    • Skin rash: petechiae, purpura
    • Musculoskeletal: arthralgias, myalgias, arthritis
    • Cardiovascular: hypertension, myocardial infarction
    • Renal: glomerulonephritis
    • Gastrointestinal: abdominal pain, ischemic colitis or mesenteric ischemia
    • Respiratory: sinusitis, hemoptysis, pulmonary hemorrhage, lung nodules or infiltrates
    • Nervous system: CNS or peripheral (mononeuritis multiplex)
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5
Q

Vasculitis

Diagnosis

A
  • Lab tests: elevated markers of inflammation (ESR, CRP)
  • Blood count: Anemia, thrombocytosis, leukocytosis
  • Gold standard for dx: biopsy demonstrating inflammatory infiltrate
  • Angiography demonstrating “beading of vessels” or aneurysms ⇒ suggestive, not confirmatory
  • Blood tests and imaging studies often not sufficient
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6
Q

Vasculitis

Treatment

A
  • Glucocorticoids
  • Immunosuppressive medications: cyclophosphamide, rituximab, azathioprine, Methotrexate
  • In some instances, plasmapheresis to remove circulating auto-Ab
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7
Q

Vasculitis

Pathological Classification

A
  • Infectious
  • Immunological
  • Unknown
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8
Q

Vasculitis

Vessel Classification

A
  • Large-Vessel Vasculitis
    • Temporal arteritis (Giant Cell Arteritis)
    • Takayasu Arteritis
  • Medium-Vessel Vasculitis
    • Kawasaki Disease
    • Polyarteritis Nodosa
  • Small-Vessel Vasculitis
    • ANCA-associated vasculitis
      • Granulomatosis w/ polyangiitis (Wegener’s granulomatosis)
      • Eosinophilic granulomatosis w/ polyangiitis (Churg-Strauss syndrome)
      • Microscopic polyangiitis
    • Immune complex vasculitis
      • IgA vasculitis (Henoch-Schonlein purpura)
      • Cryoglobulinemic vasculitis
      • Anti-glomerular basement membrane disease (anti-GBM disease)
  • Variable-vessel vasculitis
    • Behcet’s disease
    • Cogan’s syndrome
  • Single-organ vasculitis
  • Vasculitis associated w/ systemic disease
    • Lupus
    • Rheumatoid arthritis
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9
Q

Infectious Vasculitis

A

Direct invasion of vessel walls by pathogens

(syphilis, rickettsia, aspergillus)

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10
Q

Giant Cell Arteritis

Overview

A

“Temporal Arteritis”

  • Most common systemic vasculitis in adults
  • Affects large and medium-sized arteries
    • Aorta and its major branches
    • Predilection for the extracranial branches of the carotid a., especially the temporal aa.
  • Presents at mean age of 72 y (> 50y)
  • May cause blindness if not treated quickly
  • Etiology is unknown
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11
Q

Giant Cell Arteritis

Clinical Presentation

A
  • Headache
  • Pain and/or pulselessness of temporal artery
  • Jaw claudication
  • 50% have ocular symptoms
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12
Q

Giant Cell Arteritis

Associations

A
  • Associated w/ polymyalgia rheumatic: morning stiffness, pain in proximal muscles of arms and legs
  • Can be associated w/ aortitis
    • Ophthalmic arteries, retinal arteries, and ciliary arteries: medium vessels
    • Branches of the above: small vessels
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13
Q

Giant Cell Arteritis

Microscopic Appearance

A
  • Lymphocytes, macrophages and multinucleated giant cells (66%) in media
  • Fragmentation of internal elastic lamina
  • Affects short segments of the vessel
    (⊖ biopsy in up to 50%)
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14
Q

Giant Cell Arteritis

Gross Appearance

A
  • Thrombosis may be present
  • Vessel is often left w/ scarring
  • Aortic root may be dilated
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15
Q

Giant Cell Arteritis

Clinical Features

A
  • See ↑ ESR
  • Diagnosed by temporal artery biopsy
    • Biopsy shows multinucleated giant cells, granulomatous inflammation
  • Responsive to anti-inflammatory meds
    • If there is a question of temporal arteritis, treatment (glucocorticoids) should precede biopsy
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16
Q

Takayasu Arteritis

Overview

A
  • Affects large to medium-sized arteries
  • Aorta and its main branches are most affected
  • Young/middle-aged women of Asian descent (< 40 y/o)
  • Etiology is unknown
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17
Q

Takayasu Arteritis

DDx

A

Early-Onset Granulomatous Aortitis

Affects arteries of aortic arch: pulseless disease

Biopsy similar to temporal arteritis but different demographics

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18
Q

Takayasu Arteritis

Clinical Manifestations

A

Symptoms may include:

  • Fever
  • Fatigue
  • Arthralgias
  • Ocular Disturbances
  • Claudication
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19
Q

Takayasu Arteritis

Histology

A
  • Arteries become thickened and distorted sometimes leading to occlusion
  • Marked intimal thickening
  • Variable inflammatory infiltrate:
    • Mononuclear infiltrate of adventitia
    • Granulomatous inflammation involving the media
20
Q

Takayasu Arteritis

Diagnosis

A

Clinical signs include weak pulses (“pulseless disease”) and lower BP in the upper extremities

Dx is difficult and based on clinical findings in conjunction w/ radiologic evidence (angiogram or MRI)

21
Q

Kawasaki Disease

Overview

A
  • Predominantly a medium-sized vessel vasculitis
    • May affect large and small arteries as well
    • Often involves the coronary arteries (20%)
    • Aorta and veins may also be involved
  • A disease of infants and children < 4 y/o
  • Leading cause of acquired heart disease in children of Japan and North America
  • Associated w/ mucocutaneous lymph node syndrome
    • Rash, enlarged lymph nodes, “strawberry tongue”
22
Q

Kawasaki Disease

Clinical Manifestations

A

Symptoms include:

  • Fever
  • Conjunctivitis
  • Oral erosions
  • Palmar and plantar erythema
  • Desquamating skin rash
  • Lymphadenopathy
23
Q

Polyarteritis Nodosa

Overview

A
  • Necrotizing arteritis affecting medium and small arteries of the renal, cardiac and visceral circulation
    • Pulmonary vasculature is spared
  • Seen most often in young adults but may occur at any age
  • Disease course is variable and episodic
  • Associated w/ hepatitis B
  • Commonly causes aneurysms
  • ⊖ ANCA’s
24
Q

Polyarteritis Nodosa

Clinical Manifestations

A

Symptoms include:

  • Malaise
  • Fever of unknown origin (FUO)
  • Weight Loss
  • HTN
  • GI bleeding
  • Renal involvement is responsible for most fatalities
25
Q

Polyarteritis Nodosa

Pathogenesis

A
  • Most prominent at branch points
  • May affect only part of the circumference of a vessel
  • Acute phase reveals transmural inflammation rich in neutrophils w/ fibrinoid necrosis
  • Inflammation is replaced by fibrous thickening
  • Lesions of different ages may be seen in the same vessel
26
Q

ANCA-associated Vasculitis

A

“Pulmonary-Renal Syndromes”

  • Anti-neutrophil cytoplasmic Ab (ANCA) ⇒ variably associated w/ the disorders
    • Proteinase 3-ANCA (PR3) ⇒ cANCA
    • Myeloperoxidase-ANCA (MPO) ⇒ pANCA
    • Can be ANCA-⊖
  • Includes:
    • Granulomatosis w/ polyangiitis (formerly Wegener’s granulomatosis)
    • Eosinophilic granulomatosis w/ polyangiitis (formerly Churg-Strauss syndrome)
    • Microscopic polyangiitis
    • Immune complex vasculitis
    • IgA vasculitis (Henoch-Schonlein purpura)
    • Cryoglobulinemic vasculitis
    • Anti-glomerular basement membrane disease (anti-GBM disease)
27
Q

Granulomatosis w/ Polyangiitis

(Formerly Wegener’s)

Overview

A
  • Immunologic / hypersensitivity reaction
  • Affects young / middle-aged adults
  • Slight male predominance
  • 95% are c-ANCA ⊕ (proteinase3)
  • 80% fatal if untreated
28
Q

Granulomatosis w/ Polyangiitis

(Formerly Wegener’s)

Clinical Manifestions

A
  • Classic triad:
    • Necrotizing vasculitis of small to medium vessels ⇒ granuloma w/ upper respiratory tract disease (sinusitis)
    • Necrotizing granulomas of the upper and/or lower respiratory tract ⇒ pulmonary nodules/infiltrates
    • Necrotizing glomerulonephritis (often crescentic)
  • “Limited” WG affects only the respiratory tract
  • Systemic WG also affects the eyes, skin (palpable purpura), joints, nerves and occasionally the heart
29
Q

Churg-Strauss Syndrome

Overview

A

“Allergic Granulomatosis or Eosinophilic granulomatosis w/ polyangiitis”

  • Systemic vasculitis of small vessels
  • Usually presents in middle-aged adults w/ new onset or worsening asthma
  • Asthma may begin years before vasculitis
  • Eosinophilia heralds the second stage
  • Vasculitis (3rd stage) typically affects the skin, lungs, nerves and kidneys
  • Usu. ⊕ p-ANCA
30
Q

Churg-Strauss Syndrome

Clinical Manifestations

A

Signs and symptoms include:

  • Sinusitis and nasal polyps
  • Pulmonary infiltrates
  • Rashes, palpable purpura and nodules
  • Glomerulonephritis
  • Mononeuritis multiplex
31
Q

Microscopic Polyangiitis

Overview

A
  • Necrotizing vasculitis centered on arterioles, capillaries and venules
    • Involves the skin, mucous membranes, lungs, brain, heart, GI tract, kidneys and muscle
    • Necrotizing glomerulonephritis (GN) and pulmonary capillaritis are common
  • No granulomas
  • Affects all age groups and ethnic backgrounds
32
Q

Microscopic Polyangiitis

Clinical Manifestations

A
  • Glomerulonephritis (80%)
  • Weight loss (>70%)
  • Skin lesions (>60%)
  • Palpable purpura favoring dependent areas
  • Neuropathy and fever
  • Hemoptysis
  • Arthralgias and myalgias
33
Q

Microscopic Polyangiitis

Pathogenesis

A
  • Histology is similar to PAN
  • Lesions are consistent in age
  • ⊕ p-ANCA in 70% of cases
  • Specific for myeloperoxidase
34
Q

Immune Complex Vasculitis

A
  • Deposition of immunoglobulin or complement components
  • Often w/ glomerulonephritis
  • Includes:
    • IgA Vasculitis (Henoch-Schonlein Purpura)
    • Cryoglobulinemic Vasculitis
    • Anti-Glomerular Basement Membrane Disease (Anti-GBM Disease)
35
Q

IgA Vasculitis

A

“Henoch-Schonlein Purpura”

  • Triad of purpura, skin rash, abdominal pain
  • Can also cause kidney involvement (glomerulonephritis)
  • IgA deposition in small vessels
36
Q

Cryoglobulinemic Vasculitis

A
  • Associated w/ hepatitis C and lymphoproliferative diseases
  • Caused by precipitation of a cryoglobulin
    • Protein that is insoluble in reduced temperatures
  • Type I: monoclonal paraproteinemia (usually IgM)
  • Type II: immune complexes w/ monoclonal IgM (Rheumatoid Factor) and polyclonal IgG
  • Type III: immune complexes w/ polyclonal IgM (Rheumatoid Factor) and polyclonal IgG
37
Q

Anti-Glomerular Basement Membrane Disease

(Anti-GBM Disease)

A

Pulmonary-renal syndrome w/ deposition of anti-GBM Ab

38
Q

Hypocomplementemic Urticarial Vasculitis

A

Glomerulonephritis, arthritis, ocular inflammation, urticaria

39
Q

Behcet’s Disease

A
  • Recurrent oral/genital ulcers
  • Uveitis, arthritis, GI effects, CNS disease
  • Small vessel vasculitis and thrombosis (Variable-Vessel Vasculitis)
40
Q

Cogan’s Syndrome

A
  • Ocular inflammation (keratitis, uveitis, episcleritis)
  • Inner ear disease (sensorineural hearing loss and vestibular dysfunction)
  • Arteritis of varying sized vessels, valvulitis (Variable-Vessel Vasculitis)
41
Q

Single-Organ Vasculitis

A

May be an initial manifestation that later evolves into systemic disease

Cutaneous vasculitis, CNS vasculitis, Testicular vasculitis

42
Q

Vasculitis Associated w/ Systemic Disease

A

Lupus: commonly glomerulonephritis (30%) but can have vasculitis affecting the skin and central nervous system

Rheumatoid arthritis: medium vessel vasculitis, often affecting the kidneys, mutaneous ulcers, mononeuritis multiplex

43
Q

Vasculitis

Differential Dx

A

Many mimics of vasculitis

Generally mimics cause vascular occlusion/thromboembolism

  • Malignancy: elevated ESR, hypercoagulable state causing thrombosis
  • Infection: TB, endocarditis (causes a vasculitic-appearing rash, septic emboli)
  • Atrial myxoma
  • Cholesterol emboli
  • Buerger’s disease (Thromboangiitis obliterans)
44
Q

Thromboangiitis Obliterans

Overview

A

“Buerger’s Disease”

  • Acute and chronic vasculitis of medium and small arteries
  • Tibial and radial arteries are most often affected
  • May spread secondarily to veins and nerves
  • Typically affects young (20–40 y/o) male smokers
45
Q

Thromboangiitis Obliterans

Clinical Manifestations

A

Symptoms include:

  • Claudication
  • Numbness
  • Raynaud’s phenomenon
  • Gangrene

4. HDS Multisystem (43 decks)

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