1. Scleroderma & Pregnancy
Asim Iqbal
Dept. Obstetrics & Gynecology
Nishtar Hospital, Multan
Pakistan

2. Scleroderma
This Presentation is Dedicated to the Deceased Fetus of a Misfortunate Patient of Scleroderma. The Fetus Ended up in Intrauterine Death.

3. Scleroderma
Scleroderma:
It is a term which includes a heterogenous group of limited and systemic conditions causing hardening of the skin.

4. Scleroderma
Systemic sclerosis:
It is an extension of the disease process which implies involvement of both skin and other sites, particularly central internal organs.

5. 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

6. New cases / million population /year
Scleroderma
Incidence
4.5 to 12
New cases / million population /year

7. Disease with Female Preponderence
Scleroderma
Disease with Female Preponderence
Over all Male to female ratio 1:3
Reproductive age :8

8. Initiating factors are not known Numerous environmental agents (PVC)
Scleroderma
Etiology
Initiating factors are not known
Numerous environmental agents (PVC)
Drugs (Bleomycin, Pentezocine)
Defective immunoregulation
Autoantibodies
Cellular autoimmunity
Genetics, fetal cells, and viruses

11. Scleroderma
Pathogenesis:
Uncontrolled and irreversible proliferation of normal connective tissue along with striking vascular changes
Collagen
Proteoglycans
Fibronectin
Laminin

12. Macrophages Monocytes Lymphocytes Scleroderma Blood vessel
The relationship between these inflammatory cells, their mediators ( ) and subsequent fibrosis may be critical in the initial stages of scleroderma

13. Scleroderma
Clinical Features:
Fibrous thickening affects skin, muscles, joints, tendons, nervous system and certain internal organs especially esophagus, intestinal tract, lungs and kidneys

14. Skin (Affected in 90% of cases)
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

19. Gastrointestinal Tract (60% of cases)
Scleroderma
Clinical features and additional problems of pregnancy
Small bowel
Diarrhea
Possible malnutrition
Deficient peristalsis
Large bowel
Constipation
Obstruction
Perforation
Gastrointestinal Tract (60% of cases)
Esophagus
Esophageal strictures
Dysphagia

20. Kidneys (60% of Cases) Renal Failure Scleroderma Proteinurea
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

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

22. Muscle wasting and weakens Additional fatigue Tendons Friction rubs
Scleroderma
Clinical features and additional problems of pregnancy
Skeletal muscles
Muscle wasting and weakens
Additional fatigue
Tendons
Friction rubs
Contractutres

23. Heart Joints Arrythmias Polyarthralgia Conduction defects
Scleroderma
Clinical features and additional problems of pregnancy
Heart
Arrythmias
Conduction defects
Pericarditis
Congestive Cardiac failure
Joints
Polyarthralgia
Polyarthritis
Stiff painful muscles

24. 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 …….

25. Immunological tests Scleroderma 70 antibody (SCl-70)
Associated with diffuse disease
Anticentromere antibodies (ACA)
Associated with limited disease

26. Treatment is Symptomatic or the one which modifies the disease
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

27. 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

28. 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

29. Scleroderma & Pregnancy
Pregnancy outcome
n=101

30. Scleroderma & Pregnancy
Effect of Pregnancy on disease
n=101

31. 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.

33. 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

34. 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

35. 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

36. 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

37. Scleroderma & Pregnancy
Management of Pregnany
Post Natal Management:
Postnatal period should be monitored carefully as acute hypertension with renal and cardiac failure may occur.

38. Visit on the web http://www.sclero.org
Further Details
Progress in Obstetrics & Gynaecology , Vol. 8, Scleroderma and Pregnancy Authors: Carol Black , Salvatore Lupoli Editor: John Studd Churchill Livingstone
Visit on the web