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Clin Med III: MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

vasculitis is characterized by what 3 factors?

A
  1. size of blood vessel
  2. predilection of certain organ systems
  3. chracteristic pathologic features
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2
Q

vasculitis is classified by?

A

size of vessel involved

  1. large - aorta & great veesels
  2. medium - splanchnic vessels
  3. small - capillaries, arterioles, venules to lungs, kidneys, and skin
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3
Q

what two conditions could be considered a spectrum of one disease?

A
  1. polymyaglia rheumatica
  2. temporal arteries
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4
Q
  1. pain, stiffness in neck, shoulders, lower back, hips and thighs
  2. few have joint swelling
  3. MC fever, malaise, wt loss
  4. may have: trouble combing hair, putting on coat, or rising out of chair
A

polymyalgia rheumatica

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5
Q

w/u for polymyalgia rheumatica

A
  1. CBC, ESR, CRP - anemia, inflammation
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6
Q

tx polymyalgia rheumatica

A
  • prednisone
  • reeval if no impprovement in 72 h
  • MTX when tapering prednisone to control flares
  • monitor ESR
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7
Q

temporal arteritis is MC in who?

A
  • women
  • scandinavian descent
  • > 50y
  • possible FHx
  • associated with HLA-DR4
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8
Q

cause of temporal arteritis

A
  1. systemic panarateritis affecting medium and large-seize vessels
  2. proliferation of intima and fragmentation of interal elastic lamina
  3. involving +1 branches of carotid artery - MC temporal artery
  4. pathophys from ischemia
  5. antigen driven-activated T-lymphocyte macrophages and dendritic cells
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9
Q
  1. HA, scalp tenderness visual sx, jaw claudication or throat pain
  2. Fever, anemia fatigue, anorexia, weight loss, sweats arthralgias
  3. blindness possible
  4. tender temporal artery
  5. diminished pulses or bruits possible
A

temporal arteritis

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10
Q

nonclassical s/s of temporal arteritis

A

large artery involvement - aortic regurg, arm claudication, rsp tract problems, mononeuritis multiplex or FUO (chills, sweats, nml WBC)

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11
Q

w/u for temporal arteritis

A
  1. elevated ESR
  2. CBC - anemia, thrombocytosis
  3. ALP
  4. CK NOT elevated
  5. BX if ocular sx
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12
Q

tx for temporal arteritis

A
  1. prednisone
  2. ASA to reduce visual loss or stroke
  3. tocilizumab
  4. IV methylprednisolone if vision loss
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13
Q

complications of temporal arteritis

A
  1. ischemic optic neuropathy
  2. CVA, scalp or tongue infarction
  3. subclavian artery stenosis
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14
Q

pt with temporal arterities are 17-18x more likely to have what condition?

A

thoracic aortic aneurysm

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15
Q

what toxicity can be seen in 35-65% of temporal arteritis pts

A

glucocorticoid toxicity

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16
Q

what condition is caused by a multisystem necrotizing arteritis involving small and medium sized musclar arteries which involves the renal and visceral arteries?
spares lungs, can affect bronchial vessels

A

polyarteritis nodosa (PAN)

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17
Q

acute stages of PAN

A
  1. PMN leukocytes infiltrate all layers of vessel wall and perivascular areas
  2. mononuclear cells infiltrate area and lesion progresses
  3. fibrinoid necrosis
  4. healing of lesions
  5. aneurysmal dilation up to 1 cm
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18
Q

s/s of PAN

A
  • insidious onset
  • nonspecific/flu-like (hallmark): fever, abd pain, extremity pain, livdeo reicularis, mononeuritis multiplex
  • combo of mononeuritis multiplex + systemic illness
  • digital gangrene
  • MC LE ulcerations near malleoli
  • arteritis w/o GN
  • renin-mediated HTN
  • diffuse periumbilical pain - preipitated by eating (mesenteric vasculitis)
  • possible cardiac involvement, MI
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19
Q

w/u for PAN

A
  1. anemia
  2. elevated EST
  3. leukocytosis with predominance of NEUT
  4. classic PAN: (-) ANCA, low titers of RF/ANCA
  5. Test for Hep B
  6. genetic eval if childhoos onset
  7. DX: bx/angiogram - nodular lesions, painful testes, nerve/muscle
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20
Q

mgmt for PAN

A
  1. high dose CS
    - critically ill - pulse methylprednisolone
  2. cyclophosphamide
  3. MTX / azathioprine to maintain remission
  4. HBV+: glucocorticoid + anti-HBV, plasmapheresis
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21
Q

poor prognostics for PAN

A
  1. CKD w/ Cr >1.6
  2. proteinuria >1
  3. GI ischemia
  4. CNS dz
  5. cardiac involvement

Death MCC GI or CV causes

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22
Q

complications of PAN

A
  1. GI-bowel infarction, hemorrhage
  2. CV
  3. cyclophosphamide tx
  4. glucocorticord toxicity
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23
Q

granulomatous vasculitis of the upper and lower rsp tract together with GN

A

granulomatois with polyangiitis

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24
Q

hallmark of granulomatosis with polyangiitis

A

necrotizing vasculitis of small arteries and veins together with granuloma formationl intravascular or extravascular

25
Q

classic triad of granulomatosis with polyangiitis

A
  1. upper airway lesions
  2. lower airway
  3. renal
26
Q

s/s of granulomatosis with polyangiitis

A
  • severe upper rsp tract findings
  • saddle nose deformity
  • serous OM
  • subglottic tracheal stenosis
  • asx infilatrates, cough, hemoptysis, dyspnea, chest discomfort, endobronchial disease
  • eye involvement
  • papules, vesicles, palpable purpura, ulcers or SQ nodules
  • pericarditis, coronary vasculitis
  • cranial neuritis, mononeuritis multiplex, or cerebral vasculitis and/or granuloma
  • mild GN and proteinuria, hematuria, RBC casts
27
Q

dx granulomatosis with polyangiitis

A
  1. bx - necoritizing granulomatous vasculitis
  2. ESR, CRP, CBC,
  3. ANCA
28
Q

what type of bx has the highest diagnostic yield in granulomatosis with polyangiitis

A

pulm tissue

29
Q

grnaulomatosis with poly angiitis needs to be differentiated from what other vasculatides?

A
  1. midline granuloma and upper airway neoplasms
  2. cocaine induced
  3. lymphomatoid granulomatosis
30
Q

mgmt for severe granulomatosis with polyangiitis

A
  1. cyclophosphamide + prednisone
    - CBC q1-2wks
    - limit cyclophosphamide to 3-6 mo
  2. rituximab
  3. MTX/azathiprine for maintenance after cyclophosphamide
31
Q

what medication is FDA approved for non-hodgkins, CLL and RA that is used for granulomatosis with polyangiitis

32
Q

SE of rituximab

A
  • infusion reactions, severe mucocutaneous reactions and rarely progressive multifocal leukoencephalopathy
  • reactive hep B - need screening before tx
33
Q

avoid MTX in who?

A

renal insufficiency or chronic liver disease

34
Q

avoid what biologic for granulomatosis with polyangiitis

A

etanercept

35
Q

tx for non-severe granulomatosis with polyangiitis

A

MTX + glucocorticoid

36
Q
  • what tx has some benefit with sinonasal tissue
  • never used alone outisde upper airway WG
37
Q

complications with granulomatosis with polyangiitis

A
  1. renal insufficiency, hearing loss, tracheal stenosis, saddle nose deformity, chronic sinusitis
  2. kidney disease
  3. pulm disease
38
Q

What organ specific conditions do not usually respond well to systemic immunosuppressive tx

granulomatosis w/ polyangiitis

A
  • subglottic tracheal stenosis
  • endobronchial stenosis
39
Q

concerns with cyclophosphamide use?

granulomatosis with polyangiitis

A
  1. renal function
  2. infertility
  3. DVT, PE
  4. upper airway lesions should never be irradiated in WG
40
Q

necrotizing vasculitis of small/medium sized arteries and veins
MCC of pulmonary-renal syndrome

A

microscopic polyangiitis

41
Q

cause of microscopic polyangiitis?

A
  1. pauci-immune nongranulomatous necrotizing vasculitis
    - causes GN and pulmonar capillaritis
    - associated with ANCA
  2. overlaps iwth polyarteritis nodosa + granulomatosis w/ polyangiitis
42
Q

what differentiates microscopic polyangiitis vs WG?

A

microscopic has no granulomatous inflammation like WG

43
Q
  1. palpable “raised” purpura + other signs of cutaneous vasculitis - ulcers, splinters, hemorrhages, vesiculobullous lesions
  2. F, wt loss, MSK pain
  3. vasculitis neuro, mononeuritis multiplex
  4. interstitial lung fibrosis

dx?
w/u? findings?

A
  1. microscopic polyangiitis
  2. ANCA, CBC, ESR, UA, kidney bx
    - p-ANCA pattern
    - inc ESR, anemia, leukocytosis, thrombocytosis
    - inc acute phase reactants
    - microscopic hematuria, proteinuria, RBC casts in UA
    - kidney lesions: segmental, necrotizing GN, localized intravascular coagulation and intraglomerular thrombi
44
Q

mgmt for severe microscopic polyangiitis

A
  1. CS + cyclophosphamide/rituximab
  2. DC cyclophosphamide, start azathioprine, rittuximab, or MTX
  3. significant organ involvement = immunosuppressive tx

similar to granulomatosis w polyangiitis

45
Q

complications of microscpic polyangiitis

A
  1. pulm-renal syndrome
  2. vasculitis neuropathy
  3. renal insuff
46
Q

MC vasculitis in kids

47
Q

cause of HSP

A
  1. leukocytoclastic vasculitis with IgA deposits
  2. induced by URI, drugs, food, insect bites, immunizations

unknown cause

48
Q
  1. palpable purpura, polyarthralgia, GI sx, GN
    - abd pain - intussusception possible
    - purpura MC LE and butt
    - polyarthralgia MC knees and ankles
  2. myocardial involement (adults)

dx?
w/u?

A
  • HSP
  • CBC - leukocytosis, +/- eosinophilia; elevated IgA
49
Q

kidney bx of HSP?

A

segmental GN w/ crescents and meangial deposition of IgA

50
Q

mgmt of HSP

A
  1. chronic skin disease - resolves on its own (MC adults)
  2. Kids - prednisone
  3. severe - mycophenolate mofetil; most recover w/o tx
51
Q

effect of prednisone for HSP children?

A
  1. dec edema, arthralgia, abd discomfort
  2. DOES NOT:
    - dec freq of proteinuria
    - shorten dx duration or chances of recurrence
52
Q

complications of HSP

A
  1. CKD
  2. bowel obstruction
53
Q
  • an Ab directed against proteins in cytoplasmic granules of neutrophils and monocytes
  • dx WG and Microscopic Polyangiits
54
Q

2 types of ANCA

A
  1. C-ANCA (cytoplasmic)
  2. P-ANCA (perinuclear)
55
Q
  • diffuse, granular cytoplasmic staining pattern in immunofluroescence microscopy when serum ab bind to indicator neutrophil
  • very specific for WG

which type of ANCA

56
Q

produces perinuclear pattern of staining in neutrophil cytoplasm

which type of ANCA?

57
Q

limitations of ANCA?

A
  • adjunctive
  • not as definitive as tissue bx w/ WG
58
Q

sources of error that affect ANCA?

A
  • infection
  • neoplastic disease
59
Q

what other conditions can be seen with increased ANCA?

A
  1. WG
  2. microscopic polyarteritis
  3. idoapthic crescentic GN
  4. UC/CD
  5. primary sclerosis cholangitis
  6. Churg strauss vasculitis
  7. acute viral hepatitis
  8. autoimmune hepatitis

Clin Med III: MSK (14 decks)

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