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Nelson 21st Edition > Chap 183: SLE > Flashcards

Chap 183: SLE Flashcards

1
Q

characterized by multisystem inflammation and presence of circulating autoantibodies directed against self-antigens

A

SLE

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2
Q

true or false: compared with adults, children and adolescents with SLE have more severe disease and more widespread organ involvement

A

true

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3
Q

specific genetics that may contribute to increase risk for SLE

A

congenital deficiencies of C1q, C2 and C4

HLA types

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4
Q

true or false: SLE have family members, especially mothers and sisters, with SLE or other autoimmune diseases

A

true

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5
Q

what is the strongest risk factor for SLE

A

female sex

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6
Q

true or false: estrogen-containing oral contraceptives appear to induce flares of SLE

A

false

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7
Q

true or false: risk of flares may be increased in postmenopausal women receiving hormone replacement therapy

A

true

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8
Q

what virus is associated with SLE

A

EBV

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9
Q

true or false: UV light is known to trigger SLE disease activity

A

true

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10
Q

prevalence of SLE in children?

A

1-6/100,000

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11
Q

prevalence is higher in children or adults

A

adults

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12
Q

prevalence of SLE by sex

A

2-5:1 favor to female

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13
Q

childhood SLE is rare before what age

A

5yr

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14
Q

median age at diagnosis

A

11-12yr

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15
Q

what are the histologic features most suggestive of SLE

A

findings in the kidney and skin

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16
Q

finding in renal that can be indicative of increased risk of renal morbidity

A

diffuse proliferative glomerulonephritis (class IV)

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17
Q

what biopsy is helpful in establishing the diagnosis of SLE and to stage disease

A

renal biopsy

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18
Q

rash characterized on biopsy by hyperkeratosis, follicular plugging, and infiltration of mononuclear cells into the dermal-epidermal junction

A

discoid rash

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19
Q

finding specific for SLE, characterized by immunofluorescence examination of both affected and nonaffected skin may reveal deposition of immune complexes within the DEJ

A

lupus band test

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20
Q

what is the hallmark of SLE

A

generation of autoantibodies directed against self-antigen, particularly nucleic acids

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21
Q

give pathophysiology of SLE

A

patients with SLE&raquo_space; increase level of apoptosis (skin is highly susceptible with UV) + impaired ability to clear cell debris&raquo_space; prolong exposure to nucleic acid antigens&raquo_space; b cell stimulation&raquo_space; autoantibodies

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22
Q

true or false: innate and the adaptive arms of the immune system have been implicated in the dysregulation of the immune system seen in SLE

A

true

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23
Q

what does type I interferon signature means?

A

high level of IFN-alpha promotes expression of other proinflammatory cytokines and chemokines, maturation of monocytes into myeloid DCs, promotion of autoreactive B and T Cells, and loss of self tolerance

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24
Q

what percent of SLE has type I interferon signature

A

85%

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25
Q

neutrophil signature is identified in what percent in adult SLE and what is its importance

A

65%; potential biomarker for active lupus nephritis

26
Q

most common presenting complaints of children with SLE

A

fever, fatigue, hematologic abnormalities, arthralgia, and arthritis

27
Q

description of arthritis in SLE

A

present in 1st yr diagnosis; painful or painless; with stiffness in the morning; symmetric polyarthritis affecting large and small joints; tenosynovitis is present; joint erosion and other radiographic changes are rare

28
Q

symptom of renal disease in SLE

A

asymptomatic; in adolescent, can present as nephrotic syndrome and or renal failure

29
Q

true or false: neuropsychiatric complications of SLE may occur with or without active SLE

A

true

30
Q

what lab is very sensitive for SLE (95-99%) but not very specific (50%)

A

ANA

31
Q

what lab is specific for SLE (98%) but not as sensitive (40-65%)

A

anti dsDNA and anti-smith

32
Q

what are the differences of ACR and SLICC criteria in diagnosing SLE

A

hypocomplementemia (seen in SLICC; not in ACR)

in SLICC» include non scarring alopecia and positive coombs test in the absence of hemolytic anemia

33
Q

what criteria has higher sensitivity but lower specificity? SLICC or ACR

A

SLICC

SLICC: ACR

sensitivity: 93%:77%
specificity: 85%: 99%

34
Q

what are ACR criteria

A

look at page 1276

35
Q

what are SLICC criteria

A

look at page 1277

36
Q

what are the initial differential diagnosis of SLE

A

infections, malignancies, PSGN and other rheumatologic conditions; drug induced lupus

37
Q

what is often present in drug-induced SLE

A

anti-histone antibodies

38
Q

what disease is more common in drug-induced lupus

A

hepatitis

39
Q

true or false: a manifestation of drug-induced lupus resolves after withdrawal of offending medication

A

true

40
Q

true or false: complete recovery may take several months to years, requiring treatment with hydroxychloroquine, NSAIDS and or corticosteroid

A

true

41
Q

what lab correlates with disease activity especially nephritis

A

anti dsDNA

42
Q

lab which may show increased risk for raynaud phenomenon, interstitial lung disease and pulmonary hypertension

A

anti-RNP antibody

43
Q

lab: increased risk of neonatal lupus in offspring

A

anti-La antibody

44
Q

lab: increased risk for venous and arterial thrombotic events

A

antiphospholipid antibodies

45
Q

the primary treatment for all patients

A

sunscreen nd avoid exposure to UV

46
Q

role of hydroxychloroquine in SLE

A

recommended for all individuals when tolerated; treating mlid SLE; prevent flares, improves lipid profiles, improve mortality nd renal outcome

47
Q

potential toxicity of hydroxychloroquine

A

retinal deposition and subsequent vision impairment

48
Q

dose of hydroxychloroquine

A

4-5mg/kg (not be given >6.5mg/kg)

49
Q

what is the treatment mainstay for significant manifestations of SLE and work quickly to improve acute deterioration

A

corticosteroids

50
Q

dose of corticosteroids

A

high dose methylpred (30mg/kg/day (max of 1000mg/day) for 3 days then weekly pulses) or high dose oral pred 1-2mg/kg/day

51
Q

meds (3) used to treat persistent moderate SLE, including arthritis, significant cutaneous or hema involvement and pleural disease

A

methotrexate, leflunomide and azathioprine

52
Q

meds (3) appropriate for the treatment of lupus nephritis

A

cyclophosphamide, MMF, and azathioprine

53
Q

meds (2) used for significant hematologic manifestations

A

MMF and rituximab

54
Q

med reserved for most severe, potentially life-threatening SLE manifestations, such as renal, neuro and cardio

A

cyclophosphamide

55
Q

what should be considered giving if there is elevated CRP

A

statins

56
Q

med given if antiphospholipid antibody positive wihtou history of clot

A

aspirin

57
Q

med recommended throught the pregnancy of all SLE patients

A

hydroxychloroquine

58
Q

true or false: severeity of adult SLE is wors than pedia SLE

A

false (pedia SLE is worse than adult SLE

59
Q

5yr survival rate for pedia SLE

A

95%

60
Q

10yr survival rate of pedia SLE

A

80-90%

Nelson 21st Edition (6 decks)

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