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4. HDS Multisystem > Vasculitis > Flashcards

Vasculitis Flashcards

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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

Study These Flashcards
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

Study These Flashcards
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

Study These Flashcards
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

Study These Flashcards
A

Symptoms include:

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

Polyarteritis Nodosa

Overview

Study These Flashcards
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

Study These Flashcards
A

Symptoms include:

  • Malaise
  • Fever of unknown origin (FUO)
  • Weight Loss
  • HTN
  • GI bleeding
  • Renal involvement is responsible for most fatalities
25
Polyarteritis Nodosa Pathogenesis
* 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
ANCA-associated Vasculitis
“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
Granulomatosis w/ Polyangiitis (Formerly Wegener’s) Overview
* Immunologic / hypersensitivity reaction * **Affects young / middle-aged adults** * Slight male predominance * **95% are c-ANCA ⊕ (proteinase3)** * 80% fatal if untreated
28
Granulomatosis w/ Polyangiitis (Formerly Wegener’s) Clinical Manifestions
* _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
Churg-Strauss Syndrome Overview
**“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
Churg-Strauss Syndrome Clinical Manifestations
Signs and symptoms include: * **Sinusitis and nasal polyps** * **Pulmonary infiltrates** * Rashes, palpable purpura and nodules * **Glomerulonephritis** * Mononeuritis multiplex
31
Microscopic Polyangiitis Overview
* **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
Microscopic Polyangiitis Clinical Manifestations
* Glomerulonephritis (80%) * Weight loss (\>70%) * Skin lesions (\>60%) * Palpable purpura favoring dependent areas * Neuropathy and fever * Hemoptysis * Arthralgias and myalgias
33
Microscopic Polyangiitis Pathogenesis
* Histology is similar to PAN * Lesions are consistent in age * ⊕ p-ANCA in 70% of cases * Specific for myeloperoxidase
34
Immune Complex Vasculitis
* **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
IgA Vasculitis
“Henoch-Schonlein Purpura” * **Triad of purpura, skin rash, abdominal pain** * Can also cause kidney involvement (glomerulonephritis) * **IgA deposition in small vessels**
36
Cryoglobulinemic Vasculitis
* **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
Anti-Glomerular Basement Membrane Disease (Anti-GBM Disease)
Pulmonary-renal syndrome w/ deposition of anti-GBM Ab
38
Hypocomplementemic Urticarial Vasculitis
Glomerulonephritis, arthritis, ocular inflammation, urticaria
39
Behcet’s Disease
* Recurrent oral/genital ulcers * Uveitis, arthritis, GI effects, CNS disease * Small vessel vasculitis and thrombosis (Variable-Vessel Vasculitis)
40
Cogan’s Syndrome
* Ocular inflammation (keratitis, uveitis, episcleritis) * Inner ear disease (sensorineural hearing loss and vestibular dysfunction) * Arteritis of varying sized vessels, valvulitis (Variable-Vessel Vasculitis)
41
Single-Organ Vasculitis
May be an initial manifestation that later evolves into systemic disease Cutaneous vasculitis, CNS vasculitis, Testicular vasculitis
42
Vasculitis Associated w/ Systemic Disease
**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
Vasculitis Differential Dx
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
Thromboangiitis Obliterans Overview
“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
Thromboangiitis Obliterans Clinical Manifestations
Symptoms include: * Claudication * Numbness * Raynaud’s phenomenon * Gangrene

4. HDS Multisystem (43 decks)

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