Systemic Sclerosis

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Systemic sclerosis is one of the so-called 'connective tissue diseases', and affects many systems and organs. It is three times more common in women than in men, and occurs mainly in middle-aged or elderly women. The main abnormality is an excess formation of fibrous tissue in tissues.
The fibrous tissue is particularly collagenous, and leads to rigidity of the affected tissue, often with the destruction of specialized cells.
Vessel wall thickening and perivascular fibrosis are characteristic features of systemic sclerosis,
and are responsible for slowly progressive ischaemic damage in a wide range of tissues.
The skin is the most commonly affected organ in systemic sclerosis, but the alimentary tract,
lung, kidney and heart may also be involved.
Involvement of the alimentary tract in systemic sclerosis affects particularly the oesophagus
Proliferating fibrosis with collagenosis in the oesophageal muscle wall leads to the destruction of the smooth muscle, and its replacement by collagenous fibrous tissue; nerve fibres in the oesophageal wall are also damaged by the fibrosis, and there is often thickening of the blood vessels.

These changes result in a thickened, rigid oesophageal wall, which is often incapable of coordinated contraction and therefore unable to produce efficient peristalsis. As a result, reflux oesophagitis with inflammation and even ulceration are common, and these may lead to the presence of fibrous strictures.

Kidney involvement in systemic sclerosis is largely the result of vessel abnormalities and their effects on the glomeruli Kidney involvement in systemic sclerosis usually presents acutely with evidence of severe small vessel and glomerular damage. The appearances of the afferent arterioles are very similar to
those seen in malignant hypertensive disease of the kidney, with an intimal proliferation of
small arteries and arterioles leading to the almost complete occlusion of the lumen; this is also
associated with fibrinoid necrosis of some of the afferent arterioles and of portions of the
glomerular tuft. These renal changes in progressive systemic sclerosis are called acute scleroderma kidney.

Skin involvement in systemic sclerosisis called scleroderma
In systemic sclerosis, the skin involvement consists of dermal thickening through the
process of fibrous replacement of normal dermal structures. This thickening often
extends into the subcutis, and leads to gradually increasing rigidity of the skin with tightening
and atrophy of the overlying epidermis.
The lesions affect particularly the fingers and the face: the fingers becoming hard, rigid
and shiny, and the face showing a characteristic tautness of the skin with
overlying epidermal atrophy and indrawn margins of the mouth.
In the fingers there may be associated Raynaud's phenomenon, and, in old long-standing cases,
there may be calcification of the soft tissues, particularly around the finger joints
(calcinosis cutis). The dermal fibrosis leads to destruction of skin appendages, so the skin
becomes hairless and eccrine sweat ducts are damaged.
This pattern of skin involvement is usually associated with involvement of the internal
organs, but there are a few cases where the scleroderma remains localized to the skin.
The fingers in some patterns of systemic sclerosis are narrowed with tight shiny skin.
Subcutaneous calcification (calcinosis cutis) can also be seen as white spots
on the ends of the fingers.

Lung involvement in systemic sclerosis leads to extensive interstitial fibrosis and honeycomb lung.
Progressive fibrosis of the pulmonary interstitium leads to appearances that are similar to those
seen in end-stage interstitial pneumonitis.
Respiratory bronchioles, alveolar ducts and alveolar walls are progressively destroyed,
until the lung is a fibrous network that contains large cystic spaces; this is termed 'honeycomb lung'.
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