04-17 L3 SLE Flashcards

1
Q

What is the def of SLE

A
  • it is both a type II and type III hypersensitivity rxn in which bound antibody antigen pairs (immune complexes precipitate and cause further immune response.
  • autoimmunity based on interplay b/c genetic predisposition hormonal and environmental triggers that result in reduced tolerance to self tissues.
  • Immune response anomalies, alltered antigen presentation, abnormal B-cell responses, increase func of T-helper cells, abnormal cytokine production, exaggerated effector response loss of regulator T-cells or B-cells
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2
Q

SLE genetics

A
  • >40 genes have been implicated
    • Def of C1q, C2, C4
    • mutatino w/in MHC complex HLA-DR3
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3
Q

International society of n/r pathology

Class 1-6

A
  • Class I: minimal mesangial lupus nephritis
  • Class II: mesangial proliferative lupus nephritis
  • Class III: Focal lupus nephritis (<50%)
  • Class IV: Diffuse lupus (>50%)
  • Class V: membranous lupus nephritis (global or segmental subepithelial depositis)
  • Class VI: Advanced sclerosis lupsu nephritis (>90%)
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4
Q

Lupus nephritis

which class has the worse prognosis

treatment

A
  • Prognosis
    • Class 4 w/high activity index has worse prognosis
  • Treatment
    • induction: cellcept 2-3 grams or CYc
    • Maintainence: cellcept or Imuran
    • Refractory: Rituxan, tacrolimus
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5
Q

Neuropsychiatric lupus

A
  • Neurological syndromes of CNS PNS, ANS and psychiatric disorders in which other causes have been ruled out.
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6
Q

Cardiovascular disease

A
  • 50 times higher risk of CVD
    • women with SLE vs normal
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7
Q

Pulmonary disease

A
  • acute lupus pneumonits
    • diffuse avleolar hemmohage, hemoptysis, falling hct, infiltrates dyspnea
  • PH
  • PE
  • skrinking lung syndrome
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8
Q

GI

A
  • peritonities (least common serosal lingin involved)
  • bowel vascultiis
  • pancreatitis
  • protein loosing enteropathy
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9
Q
A
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10
Q

Ocular

A
  • cotton wool spots from focal ischemia (non-specific)
  • MC-plaquenial toxicity
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11
Q

Hematologic

A
  • Penia: generally due to peripheral destruction
  • Autoimmune hemolytic anemia
  • Anemia of chronic disease
  • Leucopenia
  • Lymphophenia less than 1500
  • thromboyctopenia
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12
Q

What type of serologies and complements are seen in lupus

A
  • ANA
  • dsDNA
  • low C3 & C4
  • Anti sm-specific
  • SSA/SSB
  • AntiRNP,
  • Antiribosomal P
  • Antiphospholipid
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13
Q

Name 4 treatment options for SLE

A
  • Corticosteroids
  • Topical agents
  • Antimalarials
  • Dapsone
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14
Q

SLICC classification criterion (2012)

what are the two criteria

A
  • pt statisfies 4 of teh critiera listed (including at least one clinical criterion and one immunologic criterion)
  • Pt has biosy proven nephritis compatible with SLE and with ANA or anti-dsDNA antibodies
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15
Q

What is the first line immunnomodulatory agent in mild SLE?

A
  • **Hydroxychloroquine or ‘HCQ’ **(antimalarials)
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16
Q

Pregnancy in women w/SLE

discuss contraception

factors to consider

A
  • contraception
    • need to plan pregnancy should be discussed regularly
    • best time to plan pregnancy is when SLE is quiescent for at least 6 mons
    • SLE pts are at higher risk of miscarriages, stillbirths, preeclampsia, eclamspia, IUGR, preterm labour, thrombotic and infection complications as well as have higher rate of C sectino post martum bleeding
17
Q

Pregnancy in women w/SLE

factors to consider

A
  • factors to consider
    • SLE activity (hypocomplemenemia)
    • Maternal hypertension
    • Lupus nephritis/proteinuria/renal function
    • SSA/SSB antibodies: monitoring for neonatal lupus
    • anti-phospholipid antibodies
    • Medications
18
Q

What are the 3 diagnosit criterion of

Antiphospholipid syndrome

A
  • elevated (moderate to high titers) of anticardiolipin or anti-beta 2 glycoprotein antibdies
    • pos lupus anticoagulant
  • Prior pregnancy morbidity
    • pregnacy loss of morphological normal fetus
    • 3 consecutive pregnancy losses <10 w
    • prior preterm birth (<34 w) for eclapmsia or severe pre-eclampsia or placental insufficiency
  • prior thormbosis
19
Q

Which class c drug must you continue during pregnancy?

A
  • Plaquenil