By: DR. HAMED ELLAKWA MD OB/GYN MENOUFIYA UNIVERSITY EGYPT

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SCLERODERMA AND PREGNANCY

• By
• DR. HAMED ELLAKWA
• MD OB/GYN
• MENOUFIYA UNIVERSITY
• EGYPT
• drhamedellakwa_at_yahoo.com

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Definition

• Scleroderma is a disease characterized by changes
  in the skin resulting in bound-down skin and loss
  of range of motion in the involved parts of the
  body due to contractures in the soft tissues).

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Incidence

• Scleroderma is rare (2.3-12 cases per million per
  year) but more common in women (female to male
  ratio 31).

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Incidence

• Due to the rarity of this disease and its average
  onset at 43 years of age .
• There is a relative paucity of literature
  concerning pregnancy and scleroderma..
• There have been the usual case reports of adverse
  outcomes followed by retrospective studies and a
  prospective study.

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Clinical features

• Scleroderma may be divided into
• Localized cutaneous form (morphoea) with areas of
  waxy, thickened skin, usually on the forearms and
  hands.
• Systemic sclerosis associated with Raynaud's
  phenomenon and organ involvement
• CREST syndrome (calcinosis, Raynaud's
  phenomenon, oesophageal involvement,
  sclerodactyly, telangiectasia).

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Clinical features (Cont)

• The skin in systemic sclerosis is typically bound
  down to produce sclerodactyly, beaking of the
  nose, a fixed facial expression and limitation of
  mouth opening. Skin ulceration and partial digit
  amputation are common.
• Systemic involvement usually takes the form of
  progressive fibrosis and includes the oesophagus
  most commonly (80), lungs (45), heart (40) and
  kidneys (35).

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Pathogenesis and immunology

• The aetiology is unknown.
• Theories include a contribution from
  microchimerism.
• Male cells have been detected in affected
  tissues from skin and other organs.

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Pathogenesis and immunology

• These persistent fetal cells may alternatively
  have a protective effect which could explain why
  nulli-parous women have been found to have an
  increased risk of developing scleroderma when
  compared to parous women.
• And why they have an earlier onset of the
  disease and have more pulmonary involvement and
  death than parous women.

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Pathogenesis and immunology

• There may be associated antinuclear,
  anticentromere (associated with limited cutaneous
  systemic sclerosis/CREST syndrome), antinucleolar
  or topoiso-merase I (Scl-70) antibodies
  (associated with diffuse cutaneous scleroderma
  and lung involvement). RNA-polymerase I (U3RNP)
  antibodies are associated with pulmonary
  hypertension, but this may also develop secondary
  to lung disease.

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Pregnancy

• Effect of pregnancy on scleroderma
• The prognosis for localized cutaneous scleroderma
  without organ involvement is good.
• Those with early diffuse systemic sclerosis (lt4
  years) and/or renal involvement are at risk of
  rapid overall deterioration and renal crisis
  during pregnancy.

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Pregnancy

• Effect of pregnancy on scleroderma
• Raynaud's disease tends to improve as a result of
  vasodilation and increased blood flow.
• Reflux oesophagitis may deteriorate due to
  lowered oesophageal tone.
• Those with severe pulmonary fibrosis and
  pulmonary hypertension are at high risk of
  postpartum deterioration.

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Pregnancy

• Effect of pregnancy on scleroderma
• Scleroderma renal crisis (SRC) can present
  suddenly with malignant hypertension,
  proteinuria, and a microangiopathic hemolytic
  anemia.
• Because pregnancy may contribute to induction of
  renal crisis , patients should monitor their
  blood pressure three to five times a week. This
  is particularly true in patients with diffuse
  scleroderma of recent onset who are at greater
  risk for disease complications .

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Pregnancy

• Effect of pregnancy on scleroderma
• Despite concerns about birth defects with ACE
  inhibitors, these drugs are the drugs of choice
  in SRC and should be started immediately with
  informed consent if a scleroderma patient's blood
  pressure increases.

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Effect of scleroderma on pregnancy

• Overall success rates are 70-80, but outcomes
  are improved in those without systemic disease.
  
• There is an increased risk of premature delivery.
  Late diffuse disease is associated with an
  increased risk of miscarriage.
• Pre-eclampsia, IUGR and perinatal mortality are
  risks for women with hypertension and renal
  disease.
• .

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Effect of scleroderma on pregnancy

• Venepuncture, venous access and blood pressure
  measurement may be difficult because of skin or
  blood vessel involvement.
• General anaesthesia may be complicated by
  difficult endotracheal intubation and regional
  anaesthesia may also be difficult if there is
  skin involvement on the back.

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Management

• No treatment has been shown to influence the
  progress of scleroderma and management is
  therefore symptomatic.
• Pre-pregnancy counselling is vital to inform
  women accurately about the potential risks of
  pregnancy.
• Women with early diffuse disease should be
  advised to delay pregnancy until the disease has
  stabilized.

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Management

• Pre-pregnancy assessment with formal lung
  function tests and echocardiography is
  important
• Women with multiple or severe organ involvement
  (pulmonary hypertension, severe pulmonary
  fibrosis, renal involvement) should be advised
  against pregnancy.
• Raynaud's phenomenon may be helped by heated
  gloves or nifedipine, which may be used safely
  in pregnancy.

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Management (Cont)

• Regular multidisciplinary assessment for disease
  activity and fetal well-being and blood pressure
  checks are essential.
• Although generally contraindicated in pregnancy,
  the benefits of angiotensin-inhibiting enzyme
  (ACE) inhibitors in scleroderma renal crisis
  outweigh the risks to the fetus, and their use is
  justified in this situation.

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Management (Cont)

• Early assessment by an anesthetist is advisable
  if problems with regional or general anesthesia
  are anticipated.
• Steroid treatment (including for fetal lung
  maturity) should be avoided as this may
  precipitate a renal crisis.
• ?2-agonists should be avoided for pre term labour
  because of their vasoconstrictive action.

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Management (Cont)

• During labour these measures should be
  considered
• Sclerodermatous skin changes in the extremities
  may pose a problem in positioning the patient at
  delivery. This should be obvious during prenatal
  examinations. At the time of delivery, care needs
  to be taken to keep patients warm because 90 of
  scleroderma patients have Raynaud's phenomenon.

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Management (Cont)

• The delivery room should be kept warm and the
  patient should be kept as clothed as possible.
• Intravenous fluids should be warmed prior to
  infusion.
• . Cesarean section is not contraindicated if the
  abdominal wall is involved with scleroderma, as
  long as the surgical repair is done well .
• A meticulously repaired episiotomy will heal
  better than an unplanned tear in these

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References

• 1-Luesley and Baker (2004), Obstetrics and
  Gynecology an evidence based text for MRCOG.
• 2-Nelson-Piercy (2006) ,Handbook of Obstetric
  Medicine .
• 3-Steen (1999) ,Pregnancy in women with systemic
  sclerosis.
• 4- Wayne R. Cohen (2000), Cherry Merkatz's
  Complications of Pregnancy .

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• THE END

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