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Antiphospholipid syndrome.pptx new
definition
 Antiphospholipid syndrome or antiphospholipid
  antibody syndrome (APS or APLS or) is
  an autoimmune,hypercoagulable state caused
  by antibodies against cell-membrane phospholipids that
  provokes blood clots (thrombosis) in
  both arteries and veins as well as pregnancy-related
  complications such as miscarriage, stillbirth, preterm
  delivery, or severe preeclampsia.
 The syndrome occurs due to the autoimmune production
  of antibodies against phospholipid (aPL) of the cell
  membranes.
 In particular, the disease is characterised by antibodies
  against cardiolipin(anti-cardiolipin antibodies)
  and β2 glycoprotein I
Clinical types
 . The term "primary antiphospholipid syndrome" is used
  when APS occurs in the absence of any other related
  disease.

 APS however also occurs in the context of other
  autoimmune diseases, such as systemic lupus
  erythematosus (SLE), in which case the term "secondary
  antiphospholipid syndrome" is used.

 In rare cases, APS leads to rapid organ failure due to
  generalised thrombosis; this is termed "catastrophic
  antiphospholipid syndrome" (CAPS) and is associated
  with a high risk of death.
presentations
 APS presents in two major
  ways:
       1-thrombosis (venous or
         arterial)
       2-pregnancy loss.
 Thrombocytopenia, present
  in about 20% of cases, canbe
  an important clue.
EPIDEMIOLOGY
 Race
 No defined racial predominance for primary APS has been documented,
 although SLE is more common in African American and Hispanic
 populations.

 Sex
  A female predominance has been documented, particularly for secondary
  APS. This parallels the association of APS with SLE and other connective-
  tissue diseases,

 Age
 APS is more common in young to middle-aged adults; however, it also
 manifests in children and elderly people. .[5]
EPIDEMIOLOGY
 In patients presenting with a deep venous thrombosis, up to 30%
  will have the APS.

 In a person under age 50 with a stroke, up to 46% will have APS.


 About 8% of primary APS patients later develop SLE


 In SLE patients, about 30% have anticardiolipin and about 25% have
  the lupus anticoagulant.

 The risk of venous thrombosis in a SLE patient with the lupus
  anticoagulant is 50% by 20 years after diagnosis.
PATHOGENESIS
 The pathogenesis of APS is complex and
  multifactorial, reflecting the fact that aPL bind to
  plasma proteins and to endothelial cells involved in
  multiple steps in coagulation.
 APL interfere with the activated protein C complex
  and also bind to platelets.
 They bind to endothelial cells, leading to upregulation
  of cytokines and tissue factor.
Clinical features
 Antiphospholipid syndrome (APS) is a heterogenous disorder in
  terms of clinical manifestations and range of autoantibodies.
  major clinical features may present:

 1-Vascular thrombosis
    One or more clinical episodes of arterial, venous, or small-vessel
     thrombosis .
    Thrombosis may involve the cerebral vascular system, coronary
     arteries, pulmonary system (emboli or thromboses), arterial or
     venous system in the extremities, hepatic veins, renal veins, ocular
     arteries or veins, or adrenal glands.

 2-Pregnancy morbidity
    One or more late-term (>10 weeks' gestation) spontaneous abortions
    Three or more unexplained, consecutive, spontaneous abortions
     before 10 weeks’ gestation
Clinical features
History of any of the following should raise the
examiner's suspicion for APS:
1-Thrombosis (eg, DVT/PE, MI, transient ischemic attack [TIA], or CVA,
especially if recurrent, at an earlier age, or in the absence of other known
risk factors).
2-Miscarriage (especially late trimester or recurrent) or premature birth
3-History of heart murmur or cardiac valvular vegetations
4-History of hematologic abnormalities, such as thrombocytopenia or
hemolytic anemia
5-History of nephropathy
6-Nonthrombotic neurologic symptoms, such as migraine headaches,
chorea, seizures, transverse myelitis, Guillain-Barré syndrome, or dementia
(rare)
7-Unexplained adrenal insufficiency
8-Avascular necrosis of bone in the absence of other risk factors
9-Pulmonary hypertension
CLINICAL FEATURES




  livedo reticularis, a purplish
   lacelike reticular
   pattern,especially apparent
   on the extremities.
Clinical features
 Catastrophic APS :
   is a rare presentation with multiorgan thrombi .
   Precipitants of catastrophic APS include infection,
  surgery, pregnancy,exogenous estrogen, and
  cessation of anticoagulation.
N.B:
 In the evaluation of APS, it is necessary to exclude
  genetic and acquired causes of hypercoagulability
 Thrombosis can occur even in the presence of
  thrombocytopenia
LABORATORY FEATURES
1-Lupus Anticoagulant
  only 50% of patients with LA
  have lupus.
  To confirm the presence of an
  LA, these criteria are required:
Lab features: lA
2. A sensitive activated partial thromboplastin   time
 (aPTT) is recommended for screening.


Prolongation of the clotting time must
 be due to
  an inhibitor (rather than a factor
 deficiency). This is confirmed by demonstrating
 that the prolonged clotting time does not correct
 with a 1 : 1 or 4 : 1 mix with normal plasma.
Lab features
3-Anticardiolipin
 Anticardiolipin (aCL) is actually an antibody directed
 against negatively charged phospholipids bound to beta
 2 glycoprotein I. Only medium-to-high titers of the IgG
 or IgM isotype are accepted for the classification
 criteria.

4-Anti–Beta 2 Glycoprotein I
Thus, anti-beta 2 GPI is rarely necessary to make the
 diagnosis/classifi cation of APS.
Diagnostic CRITERIA FOR ANTIPHOSPHOLIPID
     SYNDROME               (sydney revision)
 One clinical criterion
 1-Thrombosis
        Arterial Or Venous or Vasculopathy

  2-pregnancy morbidity
        a- 3 or more first trimester losses
        b- or 1 or more late fetal losses
        c- Or Severely preterm birth due to placental insufficiency
               PLUS
    One laboratory criterion: persistent over 3 months
 Lupus anticoagulant (present twice over 3 month) OR
 Moderate/high titer IgG or IgM anticardiolipin    OR
 Moderate/high titer IgG or IgM anti–beta 2 glycoprotein I

      SOURCE: From Miyakis S, et al. J Thromb Haemost 2006;4:295–306, by
        permission of Journal of Thrombosis and Haemostasis.
complications
 Antiphospholipid syndrome (APS) complications
 happen to only a minority of patients with aPL. Several
 features are thought to increase the risk of thrombosis:

1-including the lupus anticoagulant (over aCL)
2- high titers of Acl
3- persistence of aPLs for longer than 6 months,
4-comorbid factors including estrogen, thalidomide,
     nephrotic syndrome, bed rest, surgery, pregnancy,
   and the postpartum period
treatment
 Asymptomatic Antiphospholipid Antibodies

 Patients with aPL but no history of thrombosis or pregnancy
  loss
   1- should avoid medications that might contribute to
  hypercoagulability, including oral contraceptives and hormone
  therapy
  2-. Other risk factors for hypercoagulability should be
  minimized.
3- Low dose aspirin can be considered as a prophylactic
  therapy, but efficacy has not been proven in clinical trials.
4- hydroxychloroquine can be considered as a possible
  prophylactic intervention in SLE patients.
treatment
 Pregnancy Loss
  For it ‘s prevention prophylactic doses of unfractionated or low-
  molecularweight heparin plus low dose aspirin (81 mg)

This regimen causes less maternal and pregnancy morbidity than
  the older regimens of prednisone and aspirin.

Unfortunately, the heparin and aspirin regimen is successful in
 only 75% of pregnancies.

If unsuccessful, there is some scientific rationale to
the addition of intravenous immunoglobulin in the next
pregnancy.
Treatment
Thrombosis :
 The treatment of an acute thrombotic
  event (thrombolysis and/or
  heparin) .
 Because of the high risk of recurrence
  of thrombosis in APS, a strong case
  can be made for life-long
  anticoagulation after a first
  thrombotic event.
 If anticoagulation is stopped after 6
  months,there is a recurrence rate of
  20% or more .
Treatment
 The APS patient with thrombosis and thrombocytopenia
  is of special concern.
 Thrombocytopenia does not protect the APS patient from
  thrombosis.
 Most thrombocytopenia in APS is mild, in the range of 90 to
  140,000.
 Profound thrombocytopenia, however, would greatly
   increase the risk of bleeding with anticoagulation.

 The platelet count should be stable at above 50,000 before
  chronic anticoagulation is begun, and the INR goal
  would be 2.0 in such a patient.
Catastrophic Antiphospholipid
Syndrome
 treatement is by heparin, plasmapheresis or intravenous
immunoglobulin, and high dose methylprednisolone
(the latter likely calms the cytokine storm produced
by the intense endothelial cell activation) .

 Cyclophosphamide is not recommended as initial therapy
 because of the increased risk of infection.

 Mortality of catastrophic APS, even with intensive t
 reatment in major academic centers, remains 50%.
 Mortality RATE
Treatments under trials
 Statins have benefit for APS in animal models and
reduce thrombosis in clinical studies of non-APS
patients. However, they cannot be used in pregnancy
and have not been studied formally in APS.



 Rituximab depletes B cells, including B cells that
 make aPL. However, the period of B-cell depletion is
 variable, and long-lived plasma cells make aPL
  survive.
 Further studies are needed before it can be
  recommended for APS.
Antiphospholipid syndrome.pptx new
LUPUS AND MATERNAL OUTCOME

 Coexistence of other medical or obstetrical disorders.


 Whether there are an APA.


 About 40% experience flares, relatively mild in the form of
  artheralgia and rash.
PREGNANCY OUTCOME IS BETTER IF
   Lupus activity has been quiescent for at least 6
    months before conception
   There is no active renal involvement manifest by
    Proteinuria or renal dysfunction.
   Superimposed preeclampsia does not develop.
   There is no evidence of APA activity.
CONDITIONS THAT SHOULD NOT BE PREGNANT
 Active Lupus
 Nephritis, nephrotic syndrome (esp. Cr >2)
 Other organ damages (e.g. cardio-pulmonary
 involvement, anemia, thrombocytopenia, CNS
 involvement, thrombosis)
 Still take high dose steroid and immunosuppressive
 drugs
PREGNANCY COMPLICATIONS WITH SLE
1.   Preeclampsia

2. Fetal Loss

3. Preterm Delivery

4. Low Birth Weight Infant   (Glucocorticoids
     causes growth restriction)
5. Venous Thromboembolic Disease
PREPARING FOR PREGNANCY WITH SLE
 Discuss desire to have child with Rheumatologist,
 Obstetrician
 Women with Lupus nephritis are encouraged to delay
 pregnancy until their disease is inactive for at least 6
 months
MEDICATIONS DURING PREGNANCY
 Drugs to avoid (Immunosuppressant therapy)
     Mycophenolate mofetil
     Cyclophosphamide
     Methotrexate
     Biologic medications
         Etanerecpt, infliximab, anakinra
         Until more data is available, these meds should be avoided
 Drugs with small risk of harm
     Aspirin
     Prednisone/Glucocorticoids
     Azathioprine
     NSAIDs
 Drugs that are probably safe
   Antimalarials (hydroxychloroquine)
  No evidence that antimalarials increases risk of miscarriages
   or birth defects at normal doses
MANAGEMENT DURING PREGNANCY

 Assessment of severity using SLE-pregnancy disease
 activity index (SLEPDAI).
 Serial hematological studies may detect changes in
 disease activity, hemolysis, anemia, reticulocytosis,
 thrombocytopenia, leucopenia.
Antiphospholipid syndrome.pptx new
MANAGEMENT DURING PREGNANCY

 Serum transaminase activity reflects hepatic
 involvement, as does arise in serum bilirubin.
 Thrombocytopenia and Proteinuria resemble Lupus
 disease activity


 The fetus should be closely observed for adverse effects.


 Screening for anti SS-A and anti SS-B antibodies, and is
 found, fetal cardiac function should be evaluated.
RECOMMENDATIONS
 Delivery: will need stress dose steroid during active labor
 Breastfeeding: recommended even for women with SLE
 Birth control: OCP can be used but should be avoided
PHARMACOLOGICAL TREATMENT

 follow-up frequency is dependent on disease activity
 hydroxychloroquine is given to prevent flares


 Low dose aspirin is administered to prevent
  preeclampsia

 If APLS positive or history of thrombosis or fetal
  loss, treatment with heparin or LMWH and aspirin
  100 mg per day.
PHARMACOLOGICAL TREATMENT
 Severe disease is managed with corticosteroid, such as
  Prednisolone 1 to 2 mg/kg per day. or Chloroquine (250
  mg/day)


 After the disease is controlled, this is tapered to a daily
  dose of 10 to 15 mg each morning.


 Corticosteroid therapy can result in the development
  Gestational or even Type 1 Diabetes.
CORTICOSTEROID



 In patient with positive anti SSA/Ro or anti SSB
 antibodies no treatment was administered unless there
 were intrauterine cardiac complication, in which case
 Dexamethasone (4 mg/day) was given.
Neonatal Lupus syndrome
 generalized photosensitive rash
 thrombocytopenia and anemia
 giant cell hepatitis with severe cholestasis
 isolated complete heart block or cardiomyopathy
 Neonatal Lupus may appear up to 4 weeks after birth.
CONGENITAL HEART BLOCK

 The consequence of diffuse myocarditis and fibrosis in the
  region between the Atrioventricular node and Bundle of
  His
 CHB occurred almost exclusively in infants of women with
  antibodies to the SSA/Ro or SSB/La antigen causes
  unexplained stillbirth, arrhythmia is only 3%.
 The cardiac lesion is permanent, and pacemaker is
  generally necessary.
 Long term prognosis is not good, and 1/3 of affected infants
  die within 3 years.
THANK YOU!!!

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Antiphospholipid syndrome.pptx new

  • 2. definition  Antiphospholipid syndrome or antiphospholipid antibody syndrome (APS or APLS or) is an autoimmune,hypercoagulable state caused by antibodies against cell-membrane phospholipids that provokes blood clots (thrombosis) in both arteries and veins as well as pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, or severe preeclampsia.  The syndrome occurs due to the autoimmune production of antibodies against phospholipid (aPL) of the cell membranes.  In particular, the disease is characterised by antibodies against cardiolipin(anti-cardiolipin antibodies) and β2 glycoprotein I
  • 3. Clinical types  . The term "primary antiphospholipid syndrome" is used when APS occurs in the absence of any other related disease.  APS however also occurs in the context of other autoimmune diseases, such as systemic lupus erythematosus (SLE), in which case the term "secondary antiphospholipid syndrome" is used.  In rare cases, APS leads to rapid organ failure due to generalised thrombosis; this is termed "catastrophic antiphospholipid syndrome" (CAPS) and is associated with a high risk of death.
  • 4. presentations  APS presents in two major ways: 1-thrombosis (venous or arterial) 2-pregnancy loss.  Thrombocytopenia, present in about 20% of cases, canbe an important clue.
  • 5. EPIDEMIOLOGY  Race No defined racial predominance for primary APS has been documented, although SLE is more common in African American and Hispanic populations. Sex A female predominance has been documented, particularly for secondary APS. This parallels the association of APS with SLE and other connective- tissue diseases,  Age APS is more common in young to middle-aged adults; however, it also manifests in children and elderly people. .[5]
  • 6. EPIDEMIOLOGY  In patients presenting with a deep venous thrombosis, up to 30% will have the APS.  In a person under age 50 with a stroke, up to 46% will have APS.  About 8% of primary APS patients later develop SLE  In SLE patients, about 30% have anticardiolipin and about 25% have the lupus anticoagulant.  The risk of venous thrombosis in a SLE patient with the lupus anticoagulant is 50% by 20 years after diagnosis.
  • 7. PATHOGENESIS  The pathogenesis of APS is complex and multifactorial, reflecting the fact that aPL bind to plasma proteins and to endothelial cells involved in multiple steps in coagulation.  APL interfere with the activated protein C complex and also bind to platelets.  They bind to endothelial cells, leading to upregulation of cytokines and tissue factor.
  • 8. Clinical features  Antiphospholipid syndrome (APS) is a heterogenous disorder in terms of clinical manifestations and range of autoantibodies. major clinical features may present:  1-Vascular thrombosis  One or more clinical episodes of arterial, venous, or small-vessel thrombosis .  Thrombosis may involve the cerebral vascular system, coronary arteries, pulmonary system (emboli or thromboses), arterial or venous system in the extremities, hepatic veins, renal veins, ocular arteries or veins, or adrenal glands.  2-Pregnancy morbidity  One or more late-term (>10 weeks' gestation) spontaneous abortions  Three or more unexplained, consecutive, spontaneous abortions before 10 weeks’ gestation
  • 9. Clinical features History of any of the following should raise the examiner's suspicion for APS: 1-Thrombosis (eg, DVT/PE, MI, transient ischemic attack [TIA], or CVA, especially if recurrent, at an earlier age, or in the absence of other known risk factors). 2-Miscarriage (especially late trimester or recurrent) or premature birth 3-History of heart murmur or cardiac valvular vegetations 4-History of hematologic abnormalities, such as thrombocytopenia or hemolytic anemia 5-History of nephropathy 6-Nonthrombotic neurologic symptoms, such as migraine headaches, chorea, seizures, transverse myelitis, Guillain-Barré syndrome, or dementia (rare) 7-Unexplained adrenal insufficiency 8-Avascular necrosis of bone in the absence of other risk factors 9-Pulmonary hypertension
  • 10. CLINICAL FEATURES  livedo reticularis, a purplish lacelike reticular pattern,especially apparent on the extremities.
  • 11. Clinical features  Catastrophic APS : is a rare presentation with multiorgan thrombi . Precipitants of catastrophic APS include infection, surgery, pregnancy,exogenous estrogen, and cessation of anticoagulation. N.B:  In the evaluation of APS, it is necessary to exclude genetic and acquired causes of hypercoagulability  Thrombosis can occur even in the presence of thrombocytopenia
  • 12. LABORATORY FEATURES 1-Lupus Anticoagulant only 50% of patients with LA have lupus. To confirm the presence of an LA, these criteria are required:
  • 13. Lab features: lA 2. A sensitive activated partial thromboplastin time (aPTT) is recommended for screening. Prolongation of the clotting time must be due to an inhibitor (rather than a factor deficiency). This is confirmed by demonstrating that the prolonged clotting time does not correct with a 1 : 1 or 4 : 1 mix with normal plasma.
  • 14. Lab features 3-Anticardiolipin Anticardiolipin (aCL) is actually an antibody directed against negatively charged phospholipids bound to beta 2 glycoprotein I. Only medium-to-high titers of the IgG or IgM isotype are accepted for the classification criteria. 4-Anti–Beta 2 Glycoprotein I Thus, anti-beta 2 GPI is rarely necessary to make the diagnosis/classifi cation of APS.
  • 15. Diagnostic CRITERIA FOR ANTIPHOSPHOLIPID SYNDROME (sydney revision)  One clinical criterion  1-Thrombosis Arterial Or Venous or Vasculopathy 2-pregnancy morbidity a- 3 or more first trimester losses b- or 1 or more late fetal losses c- Or Severely preterm birth due to placental insufficiency PLUS One laboratory criterion: persistent over 3 months  Lupus anticoagulant (present twice over 3 month) OR  Moderate/high titer IgG or IgM anticardiolipin OR  Moderate/high titer IgG or IgM anti–beta 2 glycoprotein I SOURCE: From Miyakis S, et al. J Thromb Haemost 2006;4:295–306, by permission of Journal of Thrombosis and Haemostasis.
  • 16. complications  Antiphospholipid syndrome (APS) complications happen to only a minority of patients with aPL. Several features are thought to increase the risk of thrombosis: 1-including the lupus anticoagulant (over aCL) 2- high titers of Acl 3- persistence of aPLs for longer than 6 months, 4-comorbid factors including estrogen, thalidomide, nephrotic syndrome, bed rest, surgery, pregnancy, and the postpartum period
  • 17. treatment  Asymptomatic Antiphospholipid Antibodies Patients with aPL but no history of thrombosis or pregnancy loss 1- should avoid medications that might contribute to hypercoagulability, including oral contraceptives and hormone therapy 2-. Other risk factors for hypercoagulability should be minimized. 3- Low dose aspirin can be considered as a prophylactic therapy, but efficacy has not been proven in clinical trials. 4- hydroxychloroquine can be considered as a possible prophylactic intervention in SLE patients.
  • 18. treatment  Pregnancy Loss For it ‘s prevention prophylactic doses of unfractionated or low- molecularweight heparin plus low dose aspirin (81 mg) This regimen causes less maternal and pregnancy morbidity than the older regimens of prednisone and aspirin. Unfortunately, the heparin and aspirin regimen is successful in only 75% of pregnancies. If unsuccessful, there is some scientific rationale to the addition of intravenous immunoglobulin in the next pregnancy.
  • 19. Treatment Thrombosis :  The treatment of an acute thrombotic event (thrombolysis and/or heparin) .  Because of the high risk of recurrence of thrombosis in APS, a strong case can be made for life-long anticoagulation after a first thrombotic event.  If anticoagulation is stopped after 6 months,there is a recurrence rate of 20% or more .
  • 20. Treatment  The APS patient with thrombosis and thrombocytopenia is of special concern.  Thrombocytopenia does not protect the APS patient from thrombosis.  Most thrombocytopenia in APS is mild, in the range of 90 to 140,000.  Profound thrombocytopenia, however, would greatly increase the risk of bleeding with anticoagulation.  The platelet count should be stable at above 50,000 before chronic anticoagulation is begun, and the INR goal would be 2.0 in such a patient.
  • 21. Catastrophic Antiphospholipid Syndrome treatement is by heparin, plasmapheresis or intravenous immunoglobulin, and high dose methylprednisolone (the latter likely calms the cytokine storm produced by the intense endothelial cell activation) . Cyclophosphamide is not recommended as initial therapy because of the increased risk of infection. Mortality of catastrophic APS, even with intensive t reatment in major academic centers, remains 50%. Mortality RATE
  • 22. Treatments under trials  Statins have benefit for APS in animal models and reduce thrombosis in clinical studies of non-APS patients. However, they cannot be used in pregnancy and have not been studied formally in APS.  Rituximab depletes B cells, including B cells that make aPL. However, the period of B-cell depletion is variable, and long-lived plasma cells make aPL survive. Further studies are needed before it can be recommended for APS.
  • 24. LUPUS AND MATERNAL OUTCOME  Coexistence of other medical or obstetrical disorders.  Whether there are an APA.  About 40% experience flares, relatively mild in the form of artheralgia and rash.
  • 25. PREGNANCY OUTCOME IS BETTER IF  Lupus activity has been quiescent for at least 6 months before conception  There is no active renal involvement manifest by Proteinuria or renal dysfunction.  Superimposed preeclampsia does not develop.  There is no evidence of APA activity.
  • 26. CONDITIONS THAT SHOULD NOT BE PREGNANT  Active Lupus  Nephritis, nephrotic syndrome (esp. Cr >2)  Other organ damages (e.g. cardio-pulmonary involvement, anemia, thrombocytopenia, CNS involvement, thrombosis)  Still take high dose steroid and immunosuppressive drugs
  • 27. PREGNANCY COMPLICATIONS WITH SLE 1. Preeclampsia 2. Fetal Loss 3. Preterm Delivery 4. Low Birth Weight Infant (Glucocorticoids causes growth restriction) 5. Venous Thromboembolic Disease
  • 28. PREPARING FOR PREGNANCY WITH SLE  Discuss desire to have child with Rheumatologist, Obstetrician  Women with Lupus nephritis are encouraged to delay pregnancy until their disease is inactive for at least 6 months
  • 29. MEDICATIONS DURING PREGNANCY  Drugs to avoid (Immunosuppressant therapy)  Mycophenolate mofetil  Cyclophosphamide  Methotrexate  Biologic medications  Etanerecpt, infliximab, anakinra  Until more data is available, these meds should be avoided  Drugs with small risk of harm  Aspirin  Prednisone/Glucocorticoids  Azathioprine  NSAIDs  Drugs that are probably safe  Antimalarials (hydroxychloroquine) No evidence that antimalarials increases risk of miscarriages or birth defects at normal doses
  • 30. MANAGEMENT DURING PREGNANCY  Assessment of severity using SLE-pregnancy disease activity index (SLEPDAI).  Serial hematological studies may detect changes in disease activity, hemolysis, anemia, reticulocytosis, thrombocytopenia, leucopenia.
  • 32. MANAGEMENT DURING PREGNANCY  Serum transaminase activity reflects hepatic involvement, as does arise in serum bilirubin.  Thrombocytopenia and Proteinuria resemble Lupus disease activity  The fetus should be closely observed for adverse effects.  Screening for anti SS-A and anti SS-B antibodies, and is found, fetal cardiac function should be evaluated.
  • 33. RECOMMENDATIONS  Delivery: will need stress dose steroid during active labor  Breastfeeding: recommended even for women with SLE  Birth control: OCP can be used but should be avoided
  • 34. PHARMACOLOGICAL TREATMENT  follow-up frequency is dependent on disease activity  hydroxychloroquine is given to prevent flares  Low dose aspirin is administered to prevent preeclampsia  If APLS positive or history of thrombosis or fetal loss, treatment with heparin or LMWH and aspirin 100 mg per day.
  • 35. PHARMACOLOGICAL TREATMENT  Severe disease is managed with corticosteroid, such as Prednisolone 1 to 2 mg/kg per day. or Chloroquine (250 mg/day)  After the disease is controlled, this is tapered to a daily dose of 10 to 15 mg each morning.  Corticosteroid therapy can result in the development Gestational or even Type 1 Diabetes.
  • 36. CORTICOSTEROID  In patient with positive anti SSA/Ro or anti SSB antibodies no treatment was administered unless there were intrauterine cardiac complication, in which case Dexamethasone (4 mg/day) was given.
  • 37. Neonatal Lupus syndrome  generalized photosensitive rash  thrombocytopenia and anemia  giant cell hepatitis with severe cholestasis  isolated complete heart block or cardiomyopathy  Neonatal Lupus may appear up to 4 weeks after birth.
  • 38. CONGENITAL HEART BLOCK  The consequence of diffuse myocarditis and fibrosis in the region between the Atrioventricular node and Bundle of His  CHB occurred almost exclusively in infants of women with antibodies to the SSA/Ro or SSB/La antigen causes unexplained stillbirth, arrhythmia is only 3%.  The cardiac lesion is permanent, and pacemaker is generally necessary.  Long term prognosis is not good, and 1/3 of affected infants die within 3 years.