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SCLERODERMA
INTRODUCTION
Scleroderma, or systemic sclerosis, is a
chronic connective tissue disease generally
classified as one of the autoimmune
rheumatic diseases. The word “scleroderma”
comes from two Greek words: “sclero”
meaning hard, and “derma” meaning skin.
Hardening of the skin is one of the most
visible manifestations of the disease.
ETIOLOGY
 ENVIRONMENTAL FACTORS :
Silica dust
Organic solvents
PVC
Biogenic amines
 GENETIC PREDISPOSITION
 DEFECTIVE IMMUNOREGULATION
 Cell mediated immunity CD4/CD8,cytokines
 Humoral immunity
Hypergammaglobulinemia
Autoantibody production
Antinuclear antibody[+]>95%
•RISK FACTORS:
Genetics. People who have certain gene variations appear to be more likely
to develop scleroderma.
Environmental triggers. in some people, scleroderma symptoms may be
triggered by exposure to certain viruses, medications or drugs. Repeated
exposure — such as at work — to certain harmful substances or chemicals
also may increase the risk of scleroderma.
Immune system problems. Scleroderma is believed to be an autoimmune
disease. This means that it occurs in part because the body's immune system
begins to attack the connective tissues. In 15 to 20 percent of cases, a
person who has scleroderma also has symptoms of another autoimmune
disease, such as rheumatoid arthritis, lupus or Sjogren's syndrome.
CAUSES:
Scleroderma results from an overproduction and
accumulation of collagen in body tissues. Collagen is a
fibrous type of protein that makes up your body's
connective tissues, including your skin.
Doctors don't know exactly what causes the abnormal
collagen production to begin, but the body's immune
system appears to play a role. Most likely, scleroderma is
caused by a combination of factors, including immune
system problems, genetics and environmental triggers.
PATHOGENESIS
COMPLICATIONS:
Fingertips. The variety of Raynaud's disease that occurs with systemic
scleroderma can be so severe that the restricted blood flow
permanently damages the tissue at the fingertips, causing pits or skin
sores.
Lungs. Scarring of lung tissue can result in reduced lung function,
which can impact your ability to breathe and tolerance for exercise.
You may also develop high blood pressure in the arteries to your
lungs.
Kidneys. When scleroderma affects your kidneys, you can develop
elevated blood pressure and an increased level of protein in your
urine. More-serious effects of kidney complications may include renal
crisis, which involves a sudden increase in blood pressure and rapid
kidney failure.
Heart. Scarring of heart tissue increases your risk of abnormal heartbeats
and congestive heart failure, and can cause inflammation of the
sac surrounding your heart.
Teeth. Severe tightening of facial skin can cause your mouth to become
smaller and narrower, which may make it hard to brush your teeth or to
have them professionally cleaned. People who have scleroderma often don't
produce normal amounts of saliva, so the risk of dental decay increases
more.
Digestive system. Digestive problems associated with scleroderma can lead
to heartburn and difficulty swallowing. It can also cause bouts of cramps,
bloating, constipation or diarrhea.
Sexual function. Men who have scleroderma may experience erectile
dysfunction. Scleroderma may also affect the sexual function of women by
decreasing sexual lubrication and constricting the vaginal opening.
THANK YOU EVERYONE

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Scleroderma - A quick revision

  • 2. INTRODUCTION Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases. The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the disease.
  • 3.
  • 4. ETIOLOGY  ENVIRONMENTAL FACTORS : Silica dust Organic solvents PVC Biogenic amines  GENETIC PREDISPOSITION  DEFECTIVE IMMUNOREGULATION
  • 5.  Cell mediated immunity CD4/CD8,cytokines  Humoral immunity Hypergammaglobulinemia Autoantibody production Antinuclear antibody[+]>95%
  • 6. •RISK FACTORS: Genetics. People who have certain gene variations appear to be more likely to develop scleroderma. Environmental triggers. in some people, scleroderma symptoms may be triggered by exposure to certain viruses, medications or drugs. Repeated exposure — such as at work — to certain harmful substances or chemicals also may increase the risk of scleroderma. Immune system problems. Scleroderma is believed to be an autoimmune disease. This means that it occurs in part because the body's immune system begins to attack the connective tissues. In 15 to 20 percent of cases, a person who has scleroderma also has symptoms of another autoimmune disease, such as rheumatoid arthritis, lupus or Sjogren's syndrome.
  • 7. CAUSES: Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin. Doctors don't know exactly what causes the abnormal collagen production to begin, but the body's immune system appears to play a role. Most likely, scleroderma is caused by a combination of factors, including immune system problems, genetics and environmental triggers.
  • 9. COMPLICATIONS: Fingertips. The variety of Raynaud's disease that occurs with systemic scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores. Lungs. Scarring of lung tissue can result in reduced lung function, which can impact your ability to breathe and tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs. Kidneys. When scleroderma affects your kidneys, you can develop elevated blood pressure and an increased level of protein in your urine. More-serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
  • 10. Heart. Scarring of heart tissue increases your risk of abnormal heartbeats and congestive heart failure, and can cause inflammation of the sac surrounding your heart. Teeth. Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to have them professionally cleaned. People who have scleroderma often don't produce normal amounts of saliva, so the risk of dental decay increases more. Digestive system. Digestive problems associated with scleroderma can lead to heartburn and difficulty swallowing. It can also cause bouts of cramps, bloating, constipation or diarrhea. Sexual function. Men who have scleroderma may experience erectile dysfunction. Scleroderma may also affect the sexual function of women by decreasing sexual lubrication and constricting the vaginal opening.