What is Systemic Sclerosis (Scleroderma)?

Autoimmune rheumatic disease in the group of systemic sclerosis (scleroderma) collagen tissue diseases
is a disease. It is seen more in women than men. Genetic causes, microchimerism,
X chromosome inactivation, environmental factors, various chemicals and drugs
is responsible for its release. However, the etiopathogenesis mechanisms in general
As in rheumatic diseases, unclear medical studies continue in this regard.
is doing.

What are the signs of scleroderma?

Fibrosis in the collagen tissue (connective tissue), which generally serves as the body’s support tissue
increase is observed. The most noticeable finding of this increase is the hardening of the skin. hand fingers hand
hardening of the back, forearm, trunk, scalp and scalp attracts attention. What we sometimes call diffuse
while there is widespread skin hardening in the whole body; sometimes certain like a record or a tape.
(localized) hardening can be seen in the regions. Skin hardening is also for doctors.
It is the easiest finding to catch and track. Rank with Rodnan Leather Score(RSS)
can be determined. However, if the patient cannot be treated at a very advanced stage, the skin, subcutaneous tissue
It may appear to have softened due to atrophy. When this situation develops, it is already
Fibrotic changes in the organs have started or even settled.
Lungs are the most affected organ in internal organ involvement. gas exchange in the lungs
The inflammatory tissue, which starts from the alveolar distance from which it is made, may become fibrotic over time and
Oxygenation is disrupted as gas exchange is disrupted, and it also affects the heart.
causes the development of pulmonary hypertension (pulmonary hypertension), which is systemic
It is different from hypertension and more difficult to treat. The heart is tied to this stress
It may fail as a result of heart and lung transplantation in the long term if patients are not treated.
they can be candidates. In addition, conduction defects in the heart, impaired electrolyte exchange in the kidneys and
Development of scleroderma renal crisis with sudden and fatal rise in systemic hypertension
It can also be seen in the liver and biliary tract, pancreas, nervous system, esophagus, stomach and
intestines, in short, all organs and systems are affected by the disease.
Skin hardening causes deterioration of nutrition of the tips and especially in the tips of hands and feet.
can lead to the opening of gangrenous wounds and the development of auto-amputation. joint again

Excessive hardening of the overlying skin can lead to joint contractures and disability. in the skin
calcifications (petrifications) common, especially in contact areas, which we call ectopic
can be seen and the body tries to throw these calcifications in between, in this case, it causes skin inflammation.
why could it be.

What are the treatments for Scleroderma Patients?

Since this increase in fibrosis in the connective tissue also develops by autoimmune mechanisms, it has been previously
As we mentioned, it is one of the agents that regulate the immunosuppressive feature and cortisone.
used in treatment. Separate vasodilating agents to lower pulmonary pressure
can be given. It is important that patients reach internal diseases-rheumatology specialists, diagnosis
mapping the systemic involvement and treatment according to this map
The patient’s compliance with the treatment is very good, regular check-ups are made.
they are able to do.

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