Scleroderma and Pregnancy

Posted by e-Medical PPT
Scleroderma:It is a term which includes a heterogenous group of limited and systemic conditions causing hardening of the skin.
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

Pathogenesis
Uncontrolled and irreversible proliferation of normal connective tissue along with striking vascular changes
Collagen,Proteoglycans,Fibronectin,Laminin
The relationship between these inflammatory cells, their mediators (  ) and subsequent fibrosis may be critical in the initial stages of 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)   
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

Kidneys (60% of Cases)
Glomerular changes resulting from immune complex deposition.
Basement membrane Thickening
Mesangial hypercellularity
Intimal fibrosis of small arterioles

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

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.
Will Pregnancy be complicated ?
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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