neonatal Lupus

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NEONATAL LUPUS


Dr. Zahid Jamil
Senior Registrar/ Clinical
Fellow Neonatology
UCHS, The Childrens hospital, Lahore

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Congenital Heart Block

• A delay or interruption in the transmission of
  an impulse from atria to ventricle
• Anatomical or functional impairment diagnosed in
  fetus (in utero)
• OR
• In the first 28 days after birth (neonatal
  period).

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Types of blocks

• First degree Av Block delayed conduction from
  atrium to ventricle without interruption in atria
  to ventricle.
• Second degree AV block intermittent atrial
  conduction to ventricles in regular pattern (
  e.g. 21 , 31) further classified into
• Mobitz type I

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• Mobitz type II
• Third degree Av block No atrial impulses
  conduct to ventricle.

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ETIOLOGY
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Neonatal Lupus

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Introduction

• Passive transfer of maternal autoantibodies
  (Ro/SSA, La/SSB , Anti RnP) from mother to fetus
  results in clinical spectrum of
• Cardiac
• Cutaneous
• Systemic abnormalities
• Hematological,
• Hepatobiliary
• Pulmonary system
• Major manifestation are Cardiac and Cutaneous.
• Most serious complication is Complete heart
  Block.

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Epidemiology

• NL is rare acquired autoimmune disease that
  occurs in 1of every 20,000 births.
• The association with these specific
  autoantibodies Ro/SSA, La/SSB is independent of
  maternal disease.
• The prevalence of anti R0/SSA antibodies in
  healthy female in general population is 0.86.
• For patient with SLE, the estimated prevalence is
  40 and in Sjogren syndrome is 60 to 100.
• Approximately 50 to 60 of mothers with these
  antibodies who are asymptomatic at the time of
  babys birth, later develops symptoms consistent
  with the autoimmune disease.
• 2 of the offspring's of such mothers have
  cardiac manifestation.
• The risk of having Cutaneous disease is 4 to 16

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Pathophysiology

• Maternal antibody titer rather than their
  presence , is associated with fetal tissue
  injury.
• Genetic predisposition and environmental factors
  like viral infections may be involved.
• Transplacental passage of maternal antibodies Ro
  and La affect the developing organs particularly
  Skin and Heart.
• Injury is caused by binding of antibodies to Ro
  and La antigen which is abundant in fetal heart
  between 16 to 24 week.
• Their binding with fetal cardiocytes
• induces release of TNF by macrophages resulting
  in fibrosis and Scaring of AV node.
• inhibit Calcium Channel which are crucial to
  action potential propagation of AV node or His
  bundle.

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CLINICAL MANIFESTATIONS
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Cardiac Presentation

• Fetal presentation
• First Degree heart block
• Fetal Bradycardia
• Endocardial Fibroelastosis
• Dilated cardiomyopathy
• Myocarditis
• Neonatal presentation
• Bradycardia
• Congestive heart failure
• Intermittent canon wave in neck

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Dermatological presentation

• Erythromatous Annular Rash
• Arcuate macules with central atrophy
• Located on scalp and periorbital area.
• Histopathology resembles subacute rash of SLE

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Systemic manifestation
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Diagnosis

• The typical clinical manifestations in the
  absence of any another explanation.
• Detection of
• Anti Ro/SSA,
• Anti La /SSB
• Anti RNP antibodies in either mother or the baby
• Echocardiography

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TREATMENT
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• CARDIAC
• Close monitoring for end organ damage
• HR gt70 observe, Atropine/ Adrenaline can be given
  as per cardiac consultation
• HR lt 55 Pace maker is ultimate treatment
• CUTANEOUS
• Avoid sun exposure
• Hydroxychloroquine
• Topical low dose steroids
• HAEMATOLOGICAL
• Bone marrow suppression need IVIG (1G/kg) for
  2days and steroids (1-2mg/kg )for 5 days
• Transfusion dependency may be long term
  complication

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• RESPIRATORY
• Pneumonitis required immunosuppressive therapy
• Pulmonary hypertension with cardiac dysfunction
  can be treated with anti failure with low dose
  steroids
• HEPATIC
• Monitor Conjugated bilirubin levels and ALT twice
  weekly
• Short course steroids is recommended if portal
  fibrosis, giant cell hepatitis

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• In utero management
• First degree heart block
• Dexamethasone 4mg or betamethasone 3mg daily
• Fetal monitoring by echocardiography weekly.
• If there is progression to CHB and no extra nodal
  disease dexamethasone is discontinued.
• If block remains same or revert to SNR than
  continued to 26 week gestation than discontinued.
• Second degree hear block
• Dexamethasone 4 to 8 mg per day
• IVIG 1g/kg for 2 days
• Echocardiography Weekly
• Third degree heart block
• Treatment with isolated third degree block is not
  indicated. If Hydrops fetalis or other sign of
  fetal distress develops than emergency pacing or
  early delivery may be needed.

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Screening and Surveillance

• Prenatal maternal Screening
• Prenatal Screening for Anti R0 SSA and Anti La
  SSB is warranted in females who have
• Autoimmune disease like SLE, Sjogren syndrome
  ,rheumatoid arthritis, mixed connective tissue
  disease.
• Neonatal lupus with cutaneous or cardiac
  manifestation in previous pregnancy
• Detection of slow fetal heart rate and subsequent
  echocardiogram confirmation of heart block

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Fetal Surveillance

• Pregnancies with positive antibodies Ro/SSA and
  La/SSB need regular monitoring.
• Detection at early stage improves outcome .
• The most vulnerable period for the fetus is the
  period from 16 to 26 week of gestation
• Normal sinus rhythm can progress to congenital
  heart block during this period.
• New heart block is less likely during the 26th to
  30th week, and it rarely develops after 30 week
  of gestation.

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Fetal Monitoring

• Weekly pulsed Doppler fetal echo is advised from
  16th to 24th weeks of pregnancy.
• Fetal echocardiography enables the diagnosis of
  first degree heart block that does not result
  fetal bradycardia.

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Preventation of Neonatal Lupus in Subsequent
pregnancies

• Hydroxychloroquine.
• Should be started between 6 to 10 week of
  gestation, dose 400mg daily and maintained
  throughout pregnancy.
• Frequent monitoring should be done.
• Data(PATCH trial) suggest it may decrease risk
  of the developing fetal heart block from 18 to
  7.4.
• Preventive treatment with glucocorticoid or IVIG
  not recommended.
• If heart block detected steroid and IVIG should
  be started

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Prognosis

• Mortality and morbidity depends upon organ
  involved.
• Cutaneous lesion resolves by 6 to 9 month of age.
• Recurrence is 18 in mother with positive
  autoantibodies.
• NL with Cardiac involvement
• 50 to 60 eventually requires Pacemaker.
• Mortality 20 to 30.

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• MCQS

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MCQ 1

• Which week of Gestation is the period of highest
  risk of congenital Heart block.
• 10 to 24 weeks
• 16 to 24 weeks
• 6 to 10 weeks
• 24 to 30 weeks

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MCQ 2

• What is the reported incidence of CHB in mother
  with autoimmune disorder with known Anti Ro /SSA
  antibody.
• 2
• 8
• 18
• 24

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MCQ 3

• Cutaneous NLE can be associated with what kind of
  antibodies.
• ANA
• Anti Ro /SSA
• Anti U1RNP
• Anti La /SSB

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