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ICM TEST 2 > Vasculitides > Flashcards

Vasculitides Flashcards

1
Q

“Pulseless disease”’

  • Epidemiology (Typical patient)
  • What is it?
  • Physical findings
  • Treatment
  • Diagnostic gold standard
A

Takayasu Syndrome= LARGE VESSEL

  • younger patients (<50, asian females),
  • occlusion or narrowing of aorta/main branches
  • Physical: BP difference between extremities with bruit over subclavian/aorta
  • treat: high dose corticosteroides
  • Diagnosis: angiograph
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2
Q

Claudication of extremities

A

Takayasu

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3
Q

Young (<50) Asian female with visual and neurologic symptoms

  • Brachial pulse- absent
  • Femoral pulse- present
  • Labs- ESR elevated
  • What other symptoms might be present?
A

Takayasu- can be associated with constitutional symptoms: fever, night sweats, arthritis, arthralgias.

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4
Q

Granulomatous vasculitis

A
  • Giant cell (temporal) arteritis
  • Takayasu Arteritis
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5
Q

>50 yrs, Jaw claudication, visual loss, headache

  • Associated with?
  • Treatment?
  • Labs will show?
  • Diagnostic gold standard?
A

Temporal arteritis (systemic symptoms too),

  • [can be] associated with polymyalgia rheymatica (causing muscle pain)
  • Treatment: Medical emergency-will lead to blindness- high dose coriticosteroids
  • Diagnositc Gold standard: biopsy- need long sample. Negative dose not exclude disease
  • Labs: elevated ESR
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6
Q

Giant Cell artertitis

A
  • Older (>50), usually females
  • headache (temporal artery), visual loss (ophthalmic a), jaw claudication,
  • Systemic: Flu-like symptoms, joint and muscle pain (polymyalgia rheumatica)
  • elevated ESR
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7
Q

Necrotizing vasculitis

A

polyarteritis nodosa, buerger disease, microscopic polyangitis

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8
Q

Young adult with

  • htn,
  • abdominal pain after eating
  • Right hand wrist drop
  • palpable purpura

***What organ would NOT be involved?

A

PAN- polyarteritis nodosa- medium vessel necrotizing vasculitis

  • Htn- renal artery involvement
  • Abdominal pain- mesenteric artery- intermittent or continuous angina
  • Wrist drop- unilateral neurologic involvement
  • purpura- leukocytoclastic vasculitis

**SPARES THE LUNGS!

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9
Q

PAN

  • classic symptoms
  • serum testing might reveal?
  • Gold standard for diagnosis?
  • treatment
A

polyarteritis nodosa- small vessel necrotizing vasculitis

  • Systemic, spares the lungs
    • Renal artery invovement–> Htn
    • Mesenteric artery–> Abdominal pain (after eating), melena
    • Neuro- mono neuropathy or ASYMMETRIC polyneuropathy
    • Skin- purpura, livedo reticularis, nodules, bullous/vesicular eruptions
    • Others: Orchitis, eye, breast/uterine, spleen, MI
  • Serum: HBsAg, high BUN/creat, high APR (ESR/CRP), low serum complement, ANCA negative,
  • DIagnosis: Biopsy- gold standard, arteriogram can show stuff too
  • Treatment: corticosteroids, cyclophosphamide
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10
Q

Young child (<4 years) with MI

A

Kawasaki disease= mucocutaneous lymph node disease

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11
Q

Kawasaki Disease

  • symptoms
  • Complications
  • treatments
A

medium vessel- mucocutaneous lymph node disease

  • Symptoms: CRASH and burn
    • C- onjuntival injection
    • R-Rash (palms and soles)
    • A- adenopathy (CERVICAL)
    • S- strawberry tongue
    • H-hand/foot edema/erythema
    • Fever
  • Complications: coronary artery: aneurysm, thrombosis, MI
  • Treatment: IVIG (within 10 days of presentation to prevent aneurysm) with ASA
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12
Q

Autoamputation of toe in 35 year old male. Heavy smoker. Not diabetic

A

Beurger Disease= thromboangitits oblierans

claudication

ulceration–> gangrene–> autoamputation

Raynaud’s present

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13
Q

ANCA positive

A
  1. Microscopic polyangitis
  2. Granulomatosis with polyangitis (Wegener)
  3. Eosinophilic granulomatosis with polyangitis (Churg Strauss)
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14
Q

Middle-aged man with sinusitis and nasopharyngeal ulcer. History of hemoptosis- chest xray shows bilateral lung infiltrates that turn out to be granulomas with necrotizing vasculitis

UA- hematuria (microscopic)

  1. Diagnosis
  2. Treatment
A
  1. Wegners/Granulomatosis with polyangitis
  2. Cyclophosphamide and corticosteroids
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15
Q

Granulomatosis with polyangitis

  1. Presentation
  2. Labs
  3. Treatment
A

= Wegners

  1. nasopharyngeal ulceration/saddle nose; kidney involvement= hematuria. Chest xray with necrotizing granulomas with fixed infiltrates and cavities (snowball thing)
  2. c-anca (pr3); pauci-immune necrotizing granulomatous inflammation with necrotizing vasculitis
  3. cyclophosphamide and corticosteroids
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16
Q

Middle aged man with hoarseness, cough, and microscopic hematuria (UA). Palpable purpura

What TWO disease might this be, why is one answer better than the other? What lab result will verify it?

A

Microscopic granulomatosis vs Wegeners

  • Microscopic- no nasopharyngeal ulceration/involvement or granulomas= p-anca
  • Wegener- nasopharyngeal ulceration, or saddle nose. chest xray would show granulomas = c-anca
17
Q

Eosinophilic Granulomatosis

  1. Symptoms
  2. Lab
  3. Treatment
A
  1. Upper and lower lung, skin, CV, neuro
    • Lower lung: asthma, lung disease;
    • upper: serous otitis, allergic rhinitis, nasal polyps
    • Skin: tender subcutaneous nodules
    • CV: heart failure, pericarditis, arrythmia
    • Neuro: mononeuritis multiplex- foot drop
  2. Eosinophilia (>10%); sometimes p-anca (~40-60%); biopsy: eosinophilic infiltrates, necrotizing vasculitis or extravascular granulomas
  3. corticosteroids immunosuppresives
18
Q

Wegener/Granulomatosis with polyangitis TRIAD

A
  1. necrotizing granulomas in lung and upper airway
  2. Focal necrotizing vasculitis
  3. necrotizing glomerulonephritis
19
Q

Can cause neurological deficits like FOOT-DROP

A

Churg Strauss/ Eosinophilic granulomatosis with polyangitis

PAN- polyarteritis nodosa

20
Q

Patient has painful subcutaneous nodules, new onset of asthma, and foot-drop.

What is it? What labs might you order to confirm?

A

Churg Strauss/Eosinophilic granulomatosis with polyangitis

Bloodwork: p-anca possible, not neccesary. Eosinophilia. Biopsy would show necrotizing vasculitis with extravascular granulomas. Infiltration of vessels by eosinophils.

21
Q

Child presents with palpable purpura on legs and buttocks, history of recent URI. Low appetite.

A

HSP

IgA vasculitis- immune complex deposition. Triad: pupura, abdominal pain, arthralgias. Self-limited.

Can be associated with Berger disaese (IgA nephropathy)=hematuria, proteinuraoa.

22
Q

HSP

  1. labs
  2. treatment
A
  1. Labs:
    • UA: hematuria, white cells, casts, (no to mild) proteinuria
    • elevated IgA (50-70% of pateints)
  2. Supportive care (fluids), NSAIDs for pain. anti-htn if renal disease is bad. Usually self-limitng.
23
Q
A

ICM TEST 2 (2 decks)

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