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HemeDermMSK 2 > Vasculitides > Flashcards

Vasculitides Flashcards

1
Q

What are the three mechanisms of vasculitides?

A
  • immune complex
  • ANCA
  • granulomatous
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2
Q

By which mechanism do the following disorders cause vasculitides?

(a) SLE
(b) GPA
(c) Cryoglobulinemia
(d) Hypersensitivity
(e) EGPA, Churg-Straus
(f) Microscopic Polyangiitis
(g) Takajasu’s
(h) Henoch-Schonlein Purpura
(i) Giant Cell Artritis
(j) RA

A

Mechanisms of vasculitides

(a) SLE => immune complex
(b) GPA => granulomatous and ANCA
(c) Cryoglobulinemia = immune complex
(d) Hypersensitivity = immune complex
(e) EGPA (Churg-Straus) = ANCA associated
(f) Microscopic Polyangiitis = MPA = ANCA
(g) Takajasu’s = granulomatous
(h) Henoch-Schonlein Purpura = immune complex
(i) Giant cell arteritis = granulomatous
(j) RA = immune complex mediated

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3
Q

Name two large vessel vasculitides?

A

Giant cell arteritis and Takajasu’s

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4
Q

What is the prototypical medium vessel arteritis?

A

PAN = polyarteritis nodosa

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5
Q

Describe the mechanism of immune complex vasculitis

A

Immune complexes form when there is an excess of antigens and antibodies (often from presence of autoantibodies => Ag-Ab complexes form in circulation then deposit in tissues, or less commonly may form directly in tissues

  • immune complexes then activate complement which generates anaphylatoxins and chemoattracts for platelets and neutrophils
  • neutrophils infiltrate and release superoxide anions and lysosomal enzymes that injure tissues

Overall:
Formation of immune complexes –> ICs deposit in BV wall –> Neutrophils release lysosomal enzymes –> Fibrinoid necrosis of BV wall

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6
Q

What is ANCA?

(a) Two ways to measure presence of ANCA

A

ANCA = anti-neutrophil cytoplasmic antibodies

a) ANCA presence can be detected by indirect immunofluorescence or ELISA to specific antibodies (to PR3 or MPO

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7
Q

C-ANCA vs. P-ANCA

(a) location of fluorescent staining
(b) associated with presence of which autoantibody
(c) associated most strongly w/ which vasculitides

A

C-ANCA vs. P-ANCA

C-ANCA

(a) cytoplasmic
(b) PR-3 autoantibody
(c) strongly associated w/ GPA (granulomatous vasculitis)

P-ANCA

(a) Perinuclear
(b) Anti-MPO (myeloperoxidase) enzyme
(c) More strongly seen in EGPA and MPA

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8
Q

Describe the pathogenicity of ANCA

A
  • ANCA binds to neutrophils and monocytes, directly inducing ROS release and degranulation
  • ANCA binds to endothelial cells and induces injury
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9
Q

In ANCA vasculitides what titers can be monitored to tract treatment response and predict relapse?

A

Can monitor anti-MPO (EGPA and MPA) and anti-PR3 (GPA) antibodies to monitor treatment response and predict relapse

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10
Q

Mechanism of granulomatous vasculitis

(a) Antigen
(b) Main cells involved
(c) Key cytokine and its fxn
(d) Key histological finding

A

Granulomatous vasculitis

(a) Unknown antigen activates
(b) Th1 cells to secrete
(c) Interferon gamma which activates macrophages to form mononuclear cell infiltrates w/ epithelioid cell transformation and granuloma formation
(d) Multinucleated giant cells

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11
Q

Common presentation of a granulomatous vasculitis

A

Constitutional symptoms: fever, fatigue, weakness

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12
Q

Ddxs to rule out when considering granulomatous vasculitis

A

Infection, Tb, malignancies (mets or paraneoplastic syndrome)

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13
Q

Describe the pathology of the large vessel vasculitides

A

Large vessel vasculitides = Giant cell/temporal arteritis + Takayasu’s

Pathologically: giant cells, granulomatous inflammation, obliteration of lumen by intimal hyperplasia

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14
Q

Differ the prototypical patient (age and ethnicity) in the large vessel vasculitides

A

Giant cell arteritis: pt > 50s, Caucasian

Takayasu’s: pt

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15
Q

Distinguish the typical distribution of the two large vessel vasculitides

A

Giant cell arteritis: most commonly seen in the extracranial branches of the carotid artery (ex: temporal artery)

Takayasu’s: typically involves the aorta or aortic main branches (abdominal aorta, arch of aorta)

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16
Q

Gold standard test to diagnose giant cell arterities

(a) Other tests used

A

Gold standard test for giant cell arteritis = temporal artery biopsy

(a) Less invasive alternatives = doppler ultrasound, angiogram

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17
Q

What is polymyalgia rheumatica (PMR)?

(a) Hallmark findings
(b) Closely associated w/ which vasculitides?

A

PMR = pelvic and shoulder pain, no vasculitis if just PMR alone

(a) Hallmark findings = pelvic and shoulder pain
(b) 40-60% of giant cell arteritis cases at some point have PMR

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18
Q

What symptoms can help you diagnose GCA over PMR?

A

How to tell pt has giant cell arteritis and not just polymyalgia rheumatica?

PGA

  • unilateral headache
  • possible change/loss of vision b/c of ischemic optic neuropathy
  • jaw claudication
  • system features: fever, weakness, fatigue, wt loss

PMR
-just hip/shoulder pain w/o any of the other symptoms associated w/ PGA

19
Q

Differentiate the treatment of GCA and PMR

A

Both giant cell arteritis and polymyalgia rheumatic are responsive to steroids, but need to use very different doses (much higher in giant cell)

GCA: around 60 mg
PMR: around 15 mg

20
Q

How is Takayasu’s arteritis diagnosed?

A

Not going to biopsy the aorta…=> use imaging

-CT angiogram, MRI angiogram: shows stenosis of aortic arch etc

21
Q

2 key exam findings of Takayasu’s arteritis

A

Pts frequently present w/ arm claudication

  • decreased radial pulses
  • accelerated athosclerosis
22
Q

Treatment for Takayasu’s arteritis

(a) Adjuvant therapy

A

Takayasu’s arteritis tx = steroids
Also can use steroid sparing agents: MTX and Azothiporine (IL-6Ra)

(a) Adjuvant therapy = aspirin or anti-platelet drug b/c there’s accelerated atherosclerosis in Takayasu’s

23
Q

Why may a patient with polyarteritis nodosa have postprandial abdominal pain?

A

PAN = medium artery vasculitis w/ predilection for arterial bifurcation => ischemia of the gut => angina of the gut basically

-if arteries to the gut are blocked then can’t increase supply when needed after a meal

24
Q

What type of arteritis is most likely: 50 yo white male w/ left hand numbness and right foot drop?

A

PAN = polyarteritis nodosa- medium vessel disease

25
Q

What infectious disease is polyarteritis nodosa associated w/?

A

Chronic hep B and C

26
Q

Two consequences of vascular inflammation

A

Most common = BV occlusion => tissue ischemia

Less common = rupture of vasculature => hemorrhage into affected organ

27
Q

Differentiate the organs involved in GPA vs. MPA

(a) which one is granulomatous?
(b) Antibodies found

A

GPA (granulomatosis w/ polyangiitis) and MPA (microscopic polyangiitis)- both ANCA-associated vasculitides

GPA: lungs, kidney, ENT
MPA: just lung and kidney

(a) GPA is granulomatous
(b) GPA: anti-Pr3
MPA: anti-MPO

28
Q

Which vasculitides is staph aureus infection associated with?

A

GPA = granulomatosis w/ polyangiitis

29
Q

What is classic clinical triad of granulomatosis w/ polyangiitis?

A

Upper airway involvement (ex: sinuses, epistaxis, ansal congestion, coughing)
Lower respiratory tract (ex: granulomatous disease in lungs)
Renal

-often kidney disease is the last one in the triad to present

30
Q

Lab findings of GPA

A

C-ANCA, anti-PR3 (+), elevated ESR (sed rate)

31
Q

What is the pathological triad of granulomatosis w/ polyangiitis?

A

Granulomas
Vasculitis
Fibrinoid necrosis

^see these 3 features when you biopsy a lesion from the sinuses, lungs, or kidney

32
Q

What is the most common presenting factor of GPA?

A

GPA (granulomatosis w/ polyangiitis) most common presentation = upper airway disease

  • otologic: hearing loss
  • nasal: nasal ulcers, congestion
33
Q

Treating GPA

(a) Induction
(b) Maintenance
(c) Approved biologic

A

Treating GPA (granulomatosis w/ polyangiitis)

(a) Induce remission w/ cyclophosphamide and prednisone
(b) Maintain remission w/ MTX and Azathioprine (IL-6Ra)
(c) Approved biologic = Rituximab = anti-CD20 antibody, Rituximab is approved for all ANCA vasculitides

34
Q

What findings frequently precede a diagnosis of EGPA (eosinophilic granulomatosis w/ polyangiitis)

A

EGPA = Chugr Struass
Commonly preceded by asthma, allergy, rhinitis, and atopy (heightened immune response, tendency to develop allergic disease)

35
Q

Physical exam finding of EGPA

A

Non-blanching palpable purpura

36
Q

Lab findings of EGPA

A

Elevated white count w/ eosinophilia

P-ANCA, anti-MPO (+)

37
Q

What size vessels do the ANCA vasculitides involve?

A

Small vessels

38
Q

Diagnose the vasculitides:

  • diffuse infiltrates on CXR
  • elevated creatinine
  • nausea/vomitting
A

= MPA = microscopic polyangiitis

  • diffuse infiltrates on CXR = lung involvement
  • elevated creatinine = kidney disease
  • N/V due to uremia (kidney disease)

MPA = small vessel disease involving renal and pulmonary capillaries

39
Q

Lab findings of microscopic polyangiitis

A

MPA: P-ANCA, anti-MPO (+)

-possibly elevated creatinine or BUN indicating renal involvement

40
Q

What is the clinical triad of Behcet’s disease?

A

Oral ulcers, genital ulcers, anterior uveitis (inflammation of the uvea of the eye)

41
Q

What is Behcet’s disease?

A

Rare, immune-mediated small vessel systemic vasculitis that presents w/ mucus membrane ulceration and ocular problems

42
Q

What is Henoch Shonlein purpura?

(a) Most common in what population?
(b) Often preceded by what?

A

Systemic vasculitis (inflammation of BV) due to deposition of IgA containing immune complexes in BV walls

(a) 90% in children
(b) Often preceded by infection (ex: strep throat)

43
Q

Clinical presentation of Henoch Shonlein purpura

A

Often presents w/ joint and abdominal pain + non-blancing palpable purpura

Tetrad

  • palpable purpura (non-blanching) = mini hemorrhages
  • arthralgia
  • GI involvement
  • glomerulonephritis

HemeDermMSK 2 (11 decks)

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