Vaculitis Flashcards

1
Q

What are the types of large vessel vasculitis?

A
  • giant cell arteritis

- takayasu arteritis

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2
Q

What vessels are affected in giant cell arteritis?

A

Large & medium sized vessels of the extracranial branches of the carotid artery
Seen after 50 years of age

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3
Q

What are the predisposing factor to giant cell arteritis?

A
  • genetic predisposition
  • viral infections
  • 50% also have PMR
  • over 50 years of age
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4
Q

What is the most important pathophysiological characteristic of giant cell arteritis?

A

Focal granulomatous inflammation

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5
Q

What are the clinical features of giant cell arteritis?

A
  • constitutional symptoms
  • headaches
  • scalp tenderness
  • JAW CLAUDICATION
  • VISION LOSS
  • symptoms of PMR
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6
Q

What will be seen on lab diagnostics in case of giant cell arteritis?

A
  • increased ESR & CRP
  • TEMPORAL ARTERY BIOPSY (gold) -> mononuclear cell infiltration with giant cells
  • duplex US of temporal arteries

If suspected do NOT WAIT FOR RESULTS & TREAT IMMEDIATELY

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7
Q

How is giant cell arteritis managed?

A

High dose corticosteroids for 1-2 years titrated based on symptoms & ESR

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8
Q

What are the complications of giant cell arteritis?

A
  • vision loss

- aortic aneurysm

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9
Q

What are the vessels involved in Takayasu arteritis?

A

Pulseless aortitis & vasculitis of the large vessels & branches of the aorta

Seen in women in east asia

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10
Q

What are the symptoms of Takayasu arteritis?

A
  • fever
  • malaise
  • myalgia
  • arthralgia
  • weight loss
  • progressive claudication (in limbs)
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11
Q

How is Takayasu arteritis diagnosed & managed?

A

Diagnosed by -> angiography of aorta

Treated -> high dose corticosteroids, methotrexate, TNF inhibitors

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12
Q

What are the types of medium vessel vasculitis?

A
  • polyarteritis nodosa

- thromboangitis obliterans

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13
Q

What are the clinical features of Polyarteritis Nodosa?

A
  • fever, weight loss, malaise, muscle & joint pain
  • KIDNEY: hypertension & renal impairment
  • SKIN: livedo reticularis, nodules, ulcerations
  • neuro: mononeuropathies
  • GI: abdominal pain, melena, nausea, vomiting
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14
Q

How is PAN diagnosed?

A
  • increased ESR & CRP
    • ANCA
    • hepatitis B

Angiography -> aneurysmal dilations of affected arteries (Rosary beads) usually in renal & mesenteric

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15
Q

How is PAN treated?

A
  • high dose corticosteroids
  • cytotoxic immunosuppressive agents (cyclophosphamide)
  • antiviral therapy for HBV infection
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16
Q

What is the classic triad of thromboangitiis obliterans?

A

1- intermittent claudication
2- Raynaud phenomena
3- migratory thrombophlebitis

Usually in male smokers
Treatment is discontinuing smoking

17
Q

What are the organs involved in granulomatosis with polyangiitis?

A

Necrotizing granulomatous arteritis mostly in the kidneys & upper & lower respiratory tract

18
Q

What are the clinical features of GPA?

A
  • upper respiratory tract -> sinusitis, epistaxis, otitis media, gingivitis, stridor, mastoiditis, saddle nose
  • lungs -> cough, hemoptysis, diffuse alveolar hemorrhage, dyspnea, tracheal stenosis
  • kidneys -> rapidly progressive crescentic glomerulonephritis, hematuria
  • arthritis, scleritis, skin rashes
19
Q

How is GPA diagnosed?

A
  • ESR increased
  • C ANCA (anti proteinase 3) positive
  • biopsy -> necrotizing granulomatous
  • x ray -> pulmonary nodules or cavities
  • UA with active sedatives
20
Q

How is GPA managed?

A

INDUCTION -> cyclophosphamide + corticosteroids and/or rituximab
REMISSION -> methotrexate, azathioprine

21
Q

What are the complications of GPA?

A
  • kidney failure

- diffuse alveolar hemorrhage could be life-threatening

22
Q

What organs are most involved in eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome)?

A

small and medium vessel necrotizing vasculitis & eosinophilia most commonly in LUNGS & SKIN

23
Q

What are the clinical features of EGPA?

A
  • asthma, nasal polyps, allergic rhinitis
  • mononeuritis multiplex
  • cough, dyspnea, angina pectoris
24
Q

How is EGPA diagnosed & treated?

A
  • EOSINOPHILIA
  • MPO-ANCA & pANCA
  • XRAY -> fleeting pulmonary infiltrates
  • biopsy -> tissue eosinophilia

Treat with immunosuppression + glucocorticoids +/- cyclophosphamide

25
Q

What are the characteristics of microscopic polyangitis?

A

NONGRANULOMATOUS vasculitis of small vessels mostly in lungs, kidneys, & skin

26
Q

How is microscopic polyangitis diagnosed & treated?

A

Diagnosis

  • BIOPSY: fibrinoid necrosis with infiltration of neutrophils & no granuloma
  • MPO-ANCA & pANCA

Treatment
- immunosuppression with corticosteroids & cyclophosphamide

27
Q

What are the characteristics of Behcet disease?

A
  • oral ulcers
  • recurrent painful genital ulcerations
  • eye or cutaneous lesions
    • parhergy test

TREAT WITH systemic corticosteroids