Chap 183: SLE

Question 1

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

Answer 1

SLE

Question 2

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

Answer 2

true

Question 3

specific genetics that may contribute to increase risk for SLE

Answer 3

congenital deficiencies of C1q, C2 and C4

HLA types

Question 4

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

Answer 4

true

Question 5

what is the strongest risk factor for SLE

Answer 5

female sex

Question 6

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

Answer 6

false

Question 7

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

Answer 7

true

Question 8

what virus is associated with SLE

Answer 8

EBV

Question 9

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

Answer 9

true

Question 10

prevalence of SLE in children?

Answer 10

1-6/100,000

Question 11

prevalence is higher in children or adults

Answer 11

adults

Question 12

prevalence of SLE by sex

Answer 12

2-5:1 favor to female

Question 13

childhood SLE is rare before what age

Answer 13

5yr

Question 14

median age at diagnosis

Answer 14

11-12yr

Question 15

what are the histologic features most suggestive of SLE

Answer 15

findings in the kidney and skin

Question 16

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

Answer 16

diffuse proliferative glomerulonephritis (class IV)

Question 17

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

Answer 17

renal biopsy

Question 18

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

Answer 18

discoid rash

Question 19

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

Answer 19

lupus band test

Question 20

what is the hallmark of SLE

Answer 20

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

Question 21

give pathophysiology of SLE

Answer 21

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

Question 22

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

Answer 22

true

Question 23

what does type I interferon signature means?

Answer 23

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

Question 24

what percent of SLE has type I interferon signature

Answer 24

85%

Question 25

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

Answer 25

65%; potential biomarker for active lupus nephritis

Question 26

most common presenting complaints of children with SLE

Answer 26

fever, fatigue, hematologic abnormalities, arthralgia, and arthritis

Question 27

description of arthritis in SLE

Answer 27

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

Question 28

symptom of renal disease in SLE

Answer 28

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

Question 29

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

Answer 29

true

Question 30

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

Answer 30

ANA

Question 31

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

Answer 31

anti dsDNA and anti-smith

Question 32

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

Answer 32

hypocomplementemia (seen in SLICC; not in ACR) | in SLICC>> include non scarring alopecia and positive coombs test in the absence of hemolytic anemia

Question 33

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

Answer 33

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

Question 34

what are ACR criteria

Answer 34

look at page 1276

Question 35

what are SLICC criteria

Answer 35

look at page 1277

Question 36

what are the initial differential diagnosis of SLE

Answer 36

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

Question 37

what is often present in drug-induced SLE

Answer 37

anti-histone antibodies

Question 38

what disease is more common in drug-induced lupus

Answer 38

hepatitis

Question 39

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

Answer 39

true

Question 40

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

Answer 40

true

Question 41

what lab correlates with disease activity especially nephritis

Answer 41

anti dsDNA

Question 42

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

Answer 42

anti-RNP antibody

Question 43

lab: increased risk of neonatal lupus in offspring

Answer 43

anti-La antibody

Question 44

lab: increased risk for venous and arterial thrombotic events

Answer 44

antiphospholipid antibodies

Question 45

the primary treatment for all patients

Answer 45

sunscreen nd avoid exposure to UV

Question 46

role of hydroxychloroquine in SLE

Answer 46

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

Question 47

potential toxicity of hydroxychloroquine

Answer 47

retinal deposition and subsequent vision impairment

Question 48

dose of hydroxychloroquine

Answer 48

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

Question 49

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

Answer 49

corticosteroids

Question 50

dose of corticosteroids

Answer 50

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

Question 51

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

Answer 51

methotrexate, leflunomide and azathioprine

Question 52

meds (3) appropriate for the treatment of lupus nephritis

Answer 52

cyclophosphamide, MMF, and azathioprine

Question 53

meds (2) used for significant hematologic manifestations

Answer 53

MMF and rituximab

Question 54

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

Answer 54

cyclophosphamide

Question 55

what should be considered giving if there is elevated CRP

Answer 55

statins

Question 56

med given if antiphospholipid antibody positive wihtou history of clot

Answer 56

aspirin

Question 57

med recommended throught the pregnancy of all SLE patients

Answer 57

hydroxychloroquine

Question 58

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

Answer 58

false (pedia SLE is worse than adult SLE

Question 59

5yr survival rate for pedia SLE

Answer 59

95%

Question 60

10yr survival rate of pedia SLE

Answer 60

80-90%