Scleroderma

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Scleroderma Pregnancy
Asim Iqbal Dept. Obstetrics Gynecology Nishtar
Hospital, Multan Pakistan asimiq_at_brain.net.pk
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Scleroderma
This Presentation is Dedicated to the Deceased
Fetus of a Misfortunate Patient of Scleroderma.
The Fetus Ended up in Intrauterine Death.
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Scleroderma
Scleroderma It is a term which includes a
heterogenous group of limited and systemic
conditions causing hardening of the skin.
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Scleroderma
Systemic sclerosis It is an extension of the
disease process which implies involvement of both
skin and other sites, particularly central
internal organs.
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Scleroderma

• Types
• Localized The localized forms are Morphea and
  Linear, which affect only the skin (and sometimes
  the underlying tissues) but do not affect the
  internal organs
• Systemic The systemic forms of Scleroderma cause
  fibrosis (scar tissue) to be formed in the skin
  and/or internal organs. The fibrosis eventually
  causes the involved skin or organs to harden

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Scleroderma
Incidence
4.5 to 12
New cases / million population /year
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Scleroderma
Disease with Female Preponderence
Over all Male to female ratio 13 Reproductive
age 18
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Scleroderma
Etiology
Initiating factors are not known

• Numerous environmental agents (PVC)
• Drugs (Bleomycin, Pentezocine)
• Defective immunoregulation
• Autoantibodies
• Cellular autoimmunity
• Genetics, fetal cells, and viruses

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Scleroderma
Pathogenesis

• Uncontrolled and irreversible proliferation of
  normal connective tissue along with striking
  vascular changes
• Collagen
• Proteoglycans
• Fibronectin
• Laminin

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Scleroderma
Blood vessel

Macrophages
Monocytes
Lymphocytes
The relationship between these inflammatory
cells, their mediators ( ) and subsequent
fibrosis may be critical in the initial stages of
scleroderma
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Scleroderma
Clinical Features
Fibrous thickening affects skin, muscles, joints,
tendons, nervous system and certain internal
organs especially esophagus, intestinal tract,
lungs and kidneys
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Scleroderma
Clinical features and additional problems of
pregnancy

• Skin (Affected in 90 of cases)
• Initially, the skin is edematous, with vasculitis
  and often petechial hemorrhages.
• Enlarged vessels are frequently present
  palpable as Telengectasis
• Progressive fibrosis follows
• Flexion contractures of arms and Painful flexed
  claw like hands

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Scleroderma
Clinical features and additional problems of
pregnancy

• Small bowel
• Diarrhea
• Possible malnutrition
• Deficient peristalsis
• Large bowel
• Diarrhea
• Constipation
• Obstruction
• Perforation

Gastrointestinal Tract (60 of cases)

• Esophagus
• Esophageal strictures
• Dysphagia

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Scleroderma
Clinical features and additional problems of
pregnancy
Kidneys (60 of Cases)

• Glomerular changes resulting from immune complex
  deposition.
• Basement membrane Thickening
• Mesangial hypercellularity
• Intimal fibrosis of small arterioles

Renal Failure
Proteinurea Hypertension
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Scleroderma
Clinical features and additional problems of
pregnancy
Lungs (20 of cases)
Diffuse Interstitial Pneumonitis and
Fibrosis Honeycomb Lung
Dyspnoea Pulmonary hypertension Respiratory
Failure
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Scleroderma
Clinical features and additional problems of
pregnancy

• Skeletal muscles
• Muscle wasting and weakens
• Additional fatigue
• Tendons
• Friction rubs
• Contractutres

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Scleroderma
Clinical features and additional problems of
pregnancy

• Heart
• Arrythmias
• Conduction defects
• Pericarditis
• Congestive Cardiac failure

• Joints
• Polyarthralgia
• Polyarthritis
• Stiff painful muscles

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Scleroderma
Proper diagnosis of Scleroderma is often long and
difficult, since it is a rare disease which few
doctors are well-versed in, and in the early
stages it may resemble many other connective
tissue diseases, such as SLE, Polymyositis, and
Rheumatoid Arthritis etc .
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Scleroderma
Immunological tests
Scleroderma 70 antibody (SCl-70) Associated with
diffuse disease Anticentromere antibodies
(ACA) Associated with limited disease
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Scleroderma
Treatment
At present, there are no proven treatments or
cure for any forms of Scleroderma.

• NSAIDS
• Corticosteroids
• D-Pencillamine
• Cytotoxic drugs

Treatment is Symptomatic or the one which
modifies the disease
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Scleroderma
Anaesthetic Problems in Scleroderma

• Fibrosed skin along with vasoconstriction makes
  venous access difficult.
• Hard skin and contractures interfere with blood
  pressure and pulse oxymetric monitoring
• Difficult intubation and increased risk for
  aspiration due to esophageal sphincters
  incompetence
• Anaesthetic risks are increased due to visceral
  involvement
• Smaller doses of local anesthetics should be used
  in regional analgesia because many patients with
  Scleroderma exhibit prolonged sensory and motor
  blockade

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Scleroderma Pregnancy

• Co-occurrence of Scleroderma and pregnancy are
  unusual
• Mostly a disease of 3rd, 4th or 5th decade
• Many of these patients have infertility
• No reliable statistics for pregnancy outcome
• Mostly these are sporadic single case reports

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Scleroderma Pregnancy
Pregnancy outcome
n101
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Scleroderma Pregnancy
Effect of Pregnancy on disease
n101
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Scleroderma Pregnancy
Will Pregnancy be possible ?
It is possible that patients with Scleroderma
can achieve pregnancy although there is increased
sub-fertility in such patients.
There are conflicting reports about the increased
abortion rate.
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Scleroderma Pregnancy
Will Pregnancy be complicated ?

• It can not be predicted as available evidence is
  very limited
• Third trimester is the dangerous period with the
  risks of rapidly developing hypertension, renal
  failure and of interruption of pregnancy
• Reflux esophagitis may increase
• Small bowel involvement may cause malabsorption /
  malnutrition.
• Changes of pregnancy may cause increased
  constipation in already diseased large bowel

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Scleroderma Pregnancy
Will the baby be healthy ?
Most of the times, baby is born healthy Parents
should be informed that the risks are certainly
greater that baby might be growth effected or
born with congenital anomalies due to underlying
maternal visceral involvement and the treatment
she had been taking during pregnancy
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Scleroderma Pregnancy
Management of Pregnany

• Pre-pregnancy advice
• Ante-natal care
• Complete evaluation early in the pregnancy
• Fortnightly antenatal examination until 3rd
  trimester and thereafter weekly
• If there is evidence of Renal disease, Pulmonary
  hypertension or myocardial fibrosis - Termination
  of Pregnancy should be offered

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Scleroderma Pregnancy
Management of Pregnany

• Ante-natal care
• Symptomatic treatment for musculoskeletal
  problems
• Nutritional support
• Routine serial antenatal examination should also
  include
• Cardiopulmonary examination
• Blood Pressure monitoring
• Assessment for renal status
• Gestro-intestinal symptoms
• checks for edema

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Scleroderma Pregnancy
Management of Pregnany
Post Natal Management Postnatal period should be
monitored carefully as acute hypertension with
renal and cardiac failure may occur.
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Further Details
Progress in Obstetrics Gynaecology , Vol. 8,
1990Scleroderma and PregnancyAuthors Carol
Black , Salvatore LupoliEditor John
StuddChurchill Livingstone
Visit on the web http//www.sclero.org