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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
in middle-aged or elderly women. The main abnormality is an excess formation of
tissue in tissues.
The fibrous tissue is particularly collagenous, and leads to
of the affected tissue, often with the destruction of specialized cells.
thickening and perivascular fibrosis are characteristic features of systemic
and are responsible for slowly progressive ischaemic damage in a wide range of
The skin is the most commonly affected organ in systemic sclerosis, but the
lung, kidney and heart may also be involved.
Involvement of the alimentary tract in systemic sclerosis affects particularly
Proliferating fibrosis with collagenosis in the oesophageal muscle wall leads to
the destruction of the smooth muscle, and its replacement by collagenous fibrous
nerve fibres in the oesophageal wall are also damaged by the fibrosis, and there
thickening of the blood vessels.
These changes result in a thickened, rigid oesophageal wall, which is often
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
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
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
process of fibrous replacement of normal dermal structures. This thickening
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
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
there may be calcification of the soft tissues, particularly around the finger
(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
organs, but there are a few cases where the scleroderma remains localized to the
The fingers in some patterns of systemic sclerosis are narrowed with tight shiny
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
until the lung is a fibrous network that contains large cystic spaces; this is
termed 'honeycomb lung'.
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We give here simplified and accurate information about the disease
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