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86 Cards in this Set
- Front
- Back
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Third most common category of diseases in the US after cancer and heart disease?
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Autoimmune (80+ diseases)
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what percent of population is affected by autoimmune diseases? what % of that is women?
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5-8%
75-80% |
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Factors contributing to the development of autoimmune disorders?
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Genetic
and Environmental |
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Two types of drugs autoimmune diseases usually respond to?
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Anti-Inflammatories
Immunosuppressives |
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Who doesn't respond to Anti-Inflammatories or Immunosuppressives
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Scleroderma
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Helpful marker for SLE Dx?
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Autoantibodies directed against:
Nuclear Ag's Cytoplasmic Ag's Membrane Ag's |
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Clinical Presentation of SLE?
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Fatigue
New onset arthralgia or arthritis Serological tests (lots of false positive, but very very few false negative) |
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Public misconceptions about "autoimmune" diseases?
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Can't name one
Think HIV is one |
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Ratios of Female:Male SLE cases:
1) before puberty 2) adult years 3) after menopause |
1) 3:1
2) 10:1 3) 7-8: 1 |
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Which men are at higher risk of SLE?
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Eric
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Mechanism of SLE???
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Molecular Mimicry
Innocent Bystander Effect Disordered Apoptosis Combo of above |
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Genetics and SLE
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Most cases sporadic, but clusters of autoimmune diseases in families
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Precipitating causes of SLE exacerbations?
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Sun
UV light Infection Stress Surgery Preggers Abortion Sulfonamides NOT OCPs |
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Abnormal paps and SLE?
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Abnormal pap's are 3 times more likely in SLE
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Cancer risk and SLE?
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Significantly Elevated for Vaginal, Liver, Lung, Kidney, and several hematopoetic
Lower Risk for Breast, Cervix, and prostate |
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SLE and fertility?
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SLE doesn't affect fertility unless:
on high dose steroids w/ established renal disease and moderate to severe renal failure |
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Another SLE drug that can screw with fertility?
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Cyclophosphamide
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What affects does Cyclophosphamide have on fertility?
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Can lead to Ovarian failure (higher incidence w/ oral as opposed to IV)
Can lead to Permanent Amenorrhea in 11-59% of pts depending on age of pt and dosage |
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Contraception and SLE?
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OCPs are only ok if pt's SLE is stable or inactive and then still must consider thromboembolism or anti-phospholipid issues
Intrauterine Contraceptive Devices are ass w/ inc risk of infections |
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What is Estrogen is contraindicated in SLE pts?
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Progestins alone can be given
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Administration of Progestins?
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IM more b/c of compliance issues (q3months)
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SE's of progestins?
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Irregular Vaginal Bleeding
Acne Wt Gain Osteoporosis |
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Progestins lead to a decreased risk of?
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Ovarian Cyst Rupture
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Risks of Intrauterine Devices?
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Menstrual Bleeding
Dysmenorrhea Pelvic Infl Disease (PID) in 1st month Expulsion of Device |
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Contraindications of IUD use?
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Pregnancy
H/O Ectopic Pregnancy Curret PID Undiagnosed Vaginal Bleeding Uterine/Cervical Malignancy |
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Relative Contraindications of IUD?
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Whores
Severe Immunodeficiency Disorders Aggressive Immunosupressive Therapy |
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When is the prognosis for SLE mother and child best?
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SLE is quiescent for 6 months before pregnancy
Underlying Renal Fxn is stable and normal |
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How does SLE affect pregnancy?
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HIGH RISK PREGNANCY
Early and late miscarriages Preterm Delivery (esp w/ flare) Premature rupture of membranes Preg-induced HTN Pre-eclampsia/eclampsia SGA Stillbirth Neonatal Lupus |
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%'s of fetal loss in planned SLE pregnancies vs unplanned?
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Planned: 13%
Unplanned: 50% |
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Pre-Pregnancy Screen in mom?
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Lupus Serology: ANA, Anti-Sm, Anti-dsDNA Ab's
Serum C' levels aPLs Anti-Ro/SSA and Anti-La/SSB Ab's Routine Labs and Urinalysis Screen for major organ involvement (esp renal) |
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Lupus Flares and Pregnancy?
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Controversial if they occur more often during preggers
Can occur during all trimesters and after delivery |
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% of women who experience flares when stable for 6 months prior to preg. vs. active disease at conception?
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stable: 7-33%
active: 61-67% |
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2 major SLE complications affecting pregnancy outcome?
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Lupus nephritis
Anti-phospholipid syndrome |
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Goals of SLE Rx in relation to pregnancy?
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Maintain remission
Prophylaxis against aPL-ass thrombotic complications Prevent and Rx HTN and pre-eclampsia Optimize fetal growth |
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Safe drugs for pregnant SLE's?
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Hydroxycholoroquine
Low dose aspirin Low Dose/Therapeutic Heparin |
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Role of Hydroxychloroquine?
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Reduce Flares
Reduce Fetal Wastage Reduce SGA Reduce Fetal Distress |
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When can you give low dose aspirin?
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Only 1st and 2nd trimesters b/c it can cause a PDA in 3rd
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Why heparin?
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Prophylaxis against pre-eclampsia and thrombosis in aPL pts
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Other safe meds for SLE?
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Azathioprine
Low-dose Cyclosporin A Low-dose Steroids |
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Unsafe meds...
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Cyclophosphamide
Methotrexate Mycophenolate mofetil ACE-I's and ARBs Long acting NSAIDs Warfarin (1st and 3rd tris) |
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Risk of Pre-eclampsia in SLE pts vs non SLE pts?
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SLE: 13-32%
Normal: 3-5% |
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4 elements of Pre-Eclampsia?
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HTN
Proteinuria Edema Renal Fxn Deterioration |
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Ways to distinguish Acute Lupus Nephritis from Pre-eclampsia?
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Acute Lupus Nephritis:
Normal Liver Enzymes Low Serum C' Active Urine Sediment Inc anti-ssDNA Ab's |
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Who responds to steroids, Acute Lupus Nephritis or Pre-eclampsia?
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Lupus nephritis
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What happens in aPL syndrome?
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the aPL Ab's attach membrane phospholipids leading to venous and arterial clotting
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Who is a phospholipid that is sometimes specifically targeted (and thus testable) by Ab's?
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Cardiolipin
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% of women w/ aPLs:
normal vs undergoing IVF vs SLE |
Normal: 5%
IVF: 24% SLE: 37% |
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Impact of aPL Pregnancy?
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Stroke
Clots Preg-induced HTN Stillbirth Recurrent miscarriages SGA Preterm Birth |
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% of women w/ SLE delivering before 32 wks?
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as high as 33%
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Definition of aPL syndrome?
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Presence of aPLs w/
Vascular Thrombosis and/or recurrent abortions |
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Other features of aPL Syndrome?
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Thrombocytopenia
Neurological Disease Livedo reticularis |
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Live Birth Rate of aPL positive Lupus pts who go untreated?
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20%
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Different Rx's for aPL positive Lupus pts
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Low Dose Aspirin
Low dose aspirin + low dose heparin High dose steroids |
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Effect of low dose aspirin on live birth rate?
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Inc to 42-44%
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Effect of aspirin + heparin on live birth rate?
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Inc to 71-80%
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Effect of high dose steroids on live birth rate?
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no benefit
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5 Microangiopathic Thrombotic States?
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CAPS
DIC HELLP TMHA TTP |
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What is CAPS? extra elements?
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Catastrophic aPL Syndrome
accompanied by thrombocytopenia and hemolytic anemia |
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What is DIC?
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Disseminated Intravascular Coagulation
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what is HELLP?
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Hemolysis, Eleveated Liver Enz Levels, Low Platelet Count
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What is TMHA?
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Thrombotic Microangiopathic Hemolytic Anemia
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and TTP is?
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Thombotic Thrombocytopenia Purpura
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Risk factor for Neonatal Lupus?
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Anti-Ro/SSA and/or Anti-La/SSB Ab's
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How many SLE pts have one of these two Ab's?
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30-50%
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How many of these pts have neonates w/ Neonatal Lupus?
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5%
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how do the neonates get it?
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IgG Ab's cross the placenta
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Subsequent kids of mom who had a neonatal lupus infant have what risk in subsequent kids?
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15-25%
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Difference in risk between anti-Ro/SSA vs anti-La/SSB?
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anti-Ro/SSA: 1-2%
anti-La/SSB: 5% |
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How long does neonatal lupus usually last?
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3-6 months
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Other clinical manifestations w/ Neonatal Lupus?
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Immune Thrombocytopenia
Autoimmune Hemolytic Anemia Cholestatic hepatitis |
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Most severe complication of Neonatal Lupus?
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Congenital Heart Block
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Dx of CHB?
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Fetal bradycardia at 18-23 weeks gestation
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Mortality rate of CHB?
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20% die in neonatal period
Those that survive often require pacemakers |
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The main risk factor for CHB?
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anti-Ro/SSA or anti-La/SSB
not SLE per se |
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Risk of CHB w/ anti-Ro/SSA?
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5-7%
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Breastfeeding and SLE?
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Can be done, but premature delivery can lead to insufficient milk or suck.
Also some meds can either contaminate or suppress milk |
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Which drugs suppress milk?
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Prednisone
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Which drugs would be toxic to baby and can get into milk?
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Cytotoxic agents like:
Cyclophosphamide Azathioprine Cyclosporin A Methotrexate Big doses of aspirin should be avoided too |
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What is contraindicated in jaundiced neonates?
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NSAIDs for nursing moms
but normally are considered safe |
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A hip fracture in an osteoporosis pt does what to risk of death?
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increased by 24%
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what happens to those who survive osteoporotic hip fracture?
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50% lose their independence
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HRT and SLE Flares?
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12 months of HRT doesn't inc risk of severe flare, but doesn inc risk of mild-moderate flares
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RA and Pregnancy
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No adverse effects
Pregnancy actually helps joint sx's in 70%, but they return postpartum |
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Methotrexate and Fetuses?
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TERATOGENIC
esp in wks 6-8 Is abortive in a high dose of 50mg |
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Defects caused by methotrexate?
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craniofacial and limb
CNS (anencephaly, hydrocephaly, meningomyelocele) |
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what to do w/ methotrexate in a couple wanting to get pregnant?
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Male and/or Female discontinue use at least 3 months before conception and some would say 6 months
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