Scleroderma & Pregnancy Asim Iqbal Dept. Obstetrics & Gynecology Nishtar Hospital, Multan Pakistan asimiq@brain.net.pk

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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 …….

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

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

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

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

Scleroderma & Pregnancy Pregnancy outcome n=101

Scleroderma & Pregnancy Effect of Pregnancy on disease n=101

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.

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

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

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

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

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

Visit on the web http://www.sclero.org Further Details Progress in Obstetrics & Gynaecology , Vol. 8, 1990 Scleroderma and Pregnancy Authors: Carol Black , Salvatore Lupoli Editor: John Studd Churchill Livingstone Visit on the web http://www.sclero.org