About systemic sclerosis

What is systemic sclerosis?

Scleroderma is a rare autoimmune connective tissue disorder characterized by abnormal thickening of the skin. Connective tissue is composed of collagen, which supports and binds other body tissues. There are several types of scleroderma. Some types affect certain, specific parts of the body, while other types can affect the whole body and internal organs (systemic). Scleroderma is also known as progressive systemic sclerosis. The exact cause of scleroderma is unknown.



What are the symptoms for systemic sclerosis?

Bloating symptom was found in the systemic sclerosis condition

The symptoms and severity of the disorder vary from one person to another based on the systems and organs involved.

Skin

It is very common to experience hardening and tightening of patches of your skin with SS. The patches may be oval-shaped or a straight line that can cover large areas of your skin.

Your skin may also appear shiny since it’s so tight with your movement restricted.

Fingers and toes

Raynaud’s phenomenon results from limited cutaneous systemic scleroderma. Here, you will see small blood vessels in your fingers and toes start to constrict, or shrink, in response to cold temperatures or certain emotions.

As a result, your fingers and toes may become painful, turn blue, or go numb.

Digestive system

SS affects all or some parts of your digestive tract. If the esophagus is involved, you may experience heartburn or swallowing difficulty.

Your intestines may also be affected. In that case, you may experience:

  • cramps
  • bloating
  • diarrhea
  • constipation

In your intestines, your body absorbs the nutrients it needs to function. So if your muscles are not working correctly to digest your food, you may experience nutritional deficiencies as a result.

Heart, lungs, and kidneys

SS may affect other organs, including your heart, lungs, and kidneys. When the disease process reaches this point, it may become life-threatening without early treatment.

Scleroderma renal crisis is a rare but severe complication of SS that remains a significant risk factor for the life-threatening progression of the disease. However, a 2016 study mentioned that it only affects 2.4 to 5 percent of people who receive a diagnosis of SS.



What are the causes for systemic sclerosis?

As previously mentioned, SS occurs when your body begins to overproduce collagen and it accumulates in your tissues. Collagen is the main structural protein that makes up all of your tissues.

Doctors aren’t sure what causes the body to produce too much collagen.



What are the treatments for systemic sclerosis?

Treatment can’t cure the condition, but it can help reduce symptoms and slow disease progression. Treatment is typically based on a person’s symptoms and the need to prevent complications.

Treatment for generalized symptoms may involve:

  • corticosteroids
  • immunosuppressants, such as methotrexate or Cytoxan
  • nonsteroidal anti-inflammatory drugs (NSAIDs)

Depending on your symptoms, treatment can also include:

  • blood pressure medication
  • medication to aid breathing
  • physical or occupational therapy
  • regular dental care to prevent damage to your mouth and teeth

Treatment for Raynaud’s phenomenon may include:

  • nitroglycerine 2 percent ointment as a topical treatment
  • calcium channel blockers, like Nifedipin

You can make lifestyle changes to stay healthy with scleroderma, such as avoiding smoking cigarettes, remaining physically active, and avoiding foods that trigger heartburn.



What are the risk factors for systemic sclerosis?

Experts may not know a cause, but the immune system and a combination of other risk factors play a role.

Genetics

You may have a specific gene makeup making you more prone to developing scleroderma. Evidence suggests a higher incidence of SS in parents, siblings, and children.

The highest prevalence of SS in the United States is among a Native American tribe known as the Oklahoma Choctaw Indians, where they experience 469 cases per 100,000 people. This older statistic could be further evidence that genetics may play a significant factor in SS.

Environmental triggers

Exposure to certain agents may trigger SS, including:

  • viruses
  • medications
  • drugs
  • chemicals

Immune system issues

Since scleroderma is an autoimmune condition, it may occur because your immune system destroys your connective tissues.

An estimated 15 to 25 percent of people with SS also show signs of other connective tissue disorders, such as:

  • polymyositis
  • dermatomyositis
  • rheumatoid arthritis
  • Sjögren’s disease
  • systemic lupus erythematosus

This overlap is also known as scleroderma overlap syndrome.



Is there a cure/medications for systemic sclerosis?

The atypical growth of connective tissue caused by an autoimmune disorder is Systemic Sclerosis; the appearance and texture of skin change in this disease.
Excess immune response destroys healthy tissue as an autoimmune disease. The immune system in this disease thinks the tissue in a body is an infection.

Causes of SS:
Systemic Sclerosis is caused due to following reasons:

  • Genetics: Higher incidence of SS in parents, siblings, and children
  • Triggers from Atmosphere: Chemicals, Medications, Viruses, Drugs
  • Immune System Issues: Autoimmune condition with Connective tissue disorders that overlaps and make it Scleroderma Overlap Syndrome


Diagnosis:
Following are the tests required to confirm the disease with 100% surety:

  • Physical Examination
  • Symptoms
  • Lab tests
  • Family history of Autoimmune Diseases
  • Medical History

Basically, the disease is detected based on:

  • Skin changes
  • Symptoms
  • Review Lab Values


Cure/medications:
There are no cures or specific preventions to take to avoid the disease. But the classification is done to give the idea of how much treatment is necessary at what stage:

  • Generalised Symptoms: Prescribed medicines and regular check-ups
  • Medium Symptoms: Physical and mental aid with regular dental care
  • Complications: Treatment of Hernia, Arthritis, heart disease, intestinal obstruction, High BP, GERD, Gastroparesis, Hypertension, Pericarditis, and renal crisis
  • Raynaud’s Phenomenon: Calcium chain blockers and Topical Treatment


Symptoms
Skin Issues,Finger and Toe Issues,Digestive System Issues,Heart,Lung and kidney issues
Conditions
Oval-shaped patches on skin,Shrinking of blood vessels on skin,Heartburn,Swallow difficulty,Cramps,Bloating,Constipation
Drugs
Corticosteroids,BP medication,Immunosuppressants,Medicine to aid breathing,Anti-inflammatory drugs,Physical or occupational therapy,Regular dental care,Nitroglycerine



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