Scleroderma - Systemic Sclerosis

Hardening of the skin develops in this disorder. Systemic sclerosis is a subtype of scleroderma that affects internal organs aside from the skin. There are various symptoms that can develop from scleroderma-systemic sclerosis because the severity of the condition is different per case. To those are afflicted with this condition, regular blood and urine tests should be done. Blood pressure should also be monitored. Systemic sclerosis has no cure but treatments can alleviate the symptoms.
About two people out of 100,000 every year acquires this disorder. Women are at a higher risk than men and the condition is very rare in most children. The symptoms appear between ages thirty to sixty.
It is very possible for the woman to have a baby when she has systemic sclerosis. But the patient's pregnancy can be a high risk if there are increased complications during the pregnancy when compared to women who do not have systemic sclerosis. Obstetrician and rheumatologists can be of help to the pregnant woman. It is very important that the pregnant woman discuss her medication with her doctor before trying to conceive a child because most medicines for systemic sclerosis are not suitable for pregnant women.

Type of Scleroderma

  • Localised scleroderma or morphoea. Affects the skin only.  Some areas of the skin are harder than normal. This is common for children to have.
  • Systemic sclerosis. Also causes hardness of the skin but it may involve some of the internal organs. The two forms of this condition are:
    • diffuse cutaneous systemic sclerosis
    • limited cutaneous systemic sclerosis

Causes of Systemic Sclerosis

This is an autoimmune disorder meaning the body's own cells attack other cells inside the body. Systemic sclerosis causes damage in the connective tissue found inside the body. Systemic sclerosis is also known as connective tissue disorder. The exact mechanism how this disorder is triggered is not yet known but some suggests that fibroblast cells are infected by the immune system and makes very high amounts of collagen. Once collagen is deposited in the skin, organs and blood vessels, it causes these parts to scar and thicken or the condition called fibrosis.

Symptoms for Systemic Sclerosis

Raynaud's phenomenon is one of the earliest symptoms for this condtion. Raynaud's phenomenon happens when there are temporary changes of the skin with response to stress or coldness. This is usually found in fingers, nose and toes. The skin in these areas becomes pale, the turn blue and finally red. The color of the skin returns to normal after warming them up. Having Raynaud's phenomenon does not mean that the patient has systemic sclerosis. Systemic sclerosis occurs in one out of one hundred people with Raynaud's phenomenon.

 

Types of Systemic Sclerosis

  • Limited cutaneous systemic sclerosis. Also known as the CREST syndrome. If the patient has this syndrome then the patient also has Raynaud's phenomenon.
    • Swollen fingers
    • Tight skin
    • Patches of thickened skin on the feet, forearms and the face
  • Have ulcers in the fingertips.
  • Calcinosis forms on the skin.
  • Esophagus symptoms like acid reflux.
  • Esophageal muscles may work less which causes swallowing problems
  • Diffuse cutaneous systemic sclerosis. The progress of this type is faster. People with Raynaud's phenomenon will experience symptoms for systemic sclerosis within one year. The symptoms are worst during the first three years of the disease but after that, the disease levels off and improve the skin. The symptoms include:
    • Skin changes
    • Swelling of the skin and other body parts
    • Weight loss
    • Tiredness
    • Joint and muscle pains
    • Esophageal muscles are affected thereby causing swallowing problems.
    • Involvement of the internal organs.


Diagnosis for Systemic Sclerosis

This is difficult to diagnose because it can take on several forms and no single examination or test can give a definitive conclusion. Physical examination, blood tests and consulting a rheumatologist can also be advised.

  • Examination of fingernails to look for capillaries in the nail which can show changes due to systemic sclerosis.
  • Skin biopsy
  • Blood tests to check for antibodies that are due to systemic sclerosis.

Complications for Systemic Sclerosis

The condition can cause a lot of complications and is able to affect the internal organs in the body. This is because this condition is also called the connective tissue disease which is found throughout the body. The complications can vary from mild to severe.
The most common symptoms are the following:

  • Skin becomes tight
  • Appearance of ulcers and nodules on the skin
  • Intestines will become underactive which causes constipation and bloating
  • Muscles and joint pain can occur
  • Thyroid gland becomes underactive
  • Reduced production of saliva causes dryness of the mouth
  • Blood pressure becomes high
  • Lungs develop 'pulmonary hypertension' or having high blood pressure in the lungs and 'pulmonary fibrosis' or scarring of the tissue in the lungs
  • Kidney problems
  • 'Scleroderma renal crisis' and 'accelerated hypertension' happens when the kidney deteriorates quickly. Treatment should be given immediately. This causes breathlessness, swelling of the foot and leg, blurred vision, reduced urine production headaches and seizures

Treatment for Systemic Sclerosis

There is no known cure for this condition. The aim of the treatment is to reduce the symptoms to a tolerable level, detect any complications as early as possible, minimize disability and prevent the disorder from progressing any further.

  • To relieve symptoms.
    • Skin moisturisers prescribed by a dermatologist and special baths an help tight or dry skin
    • Raynaud's phenomenon can be reduced by using calcium antagomists, extra warmth and prostacyclin
    • Gut and stomach problems are relieved by omeprazole which reduces acid secretion. Some use domperidone to aid stomach action and some laxatives
    • Drinking lots of fluid can aid swallowing problems
  • Prevention of the condition from progressing. Treatments used suppress the immune system from overactivity because of systemic sclerosis. Tsroid, penicillamine, methotrexate, cyclophosphamide, infliximab azathioprine and stem cell transplantation can be used for treatment. The patient should also refrain from smoking because the lungs and the blood vessels need to be healthy.

 

  • Detect and treating complications early. Regular observation of the patient should be done especially to monitor his blood pressure and the function of the kidneys. Urine and blood tests are used to check for these areas while a scan is used to check for the condition of the lungs. These tests can help in the early detection of the symptoms and this would allow the patient for some early treatment.
    • Treatment for the various complications:
      • Skin – antibiotics and dressings can be used to treat skin ulcers. Nodules, ulcers and tight skin are aided by surgery.
      • Lungs – bosentan, Viagra and prostacyclin can treat pulmonary fibrosis and pulmonary hypertension
      • Kidney and high blood pressure problems can be treated using ACE inhibitors or angiotensin receptor blockers or nifedipine.
      • Thyroid replacement tablets are given to patients to control the inactivity of the thyroid.
      • People with systemic sclerosis are also prone to infections so it is normal for the doctors to prescribe antibiotics to treat infection.

 

  • Minimizing disability.
    • Physiotherapists advise on the type of exercises the patient should perform to relieve him of muscle and joint pains.
    • Occupational therapists advise on the type of exercises to be done to support the muscles and joints to help the patient perform daily tasks.
    • Dental check-ups should also be done regularly to check if the patient has dry mouth.
    • Support groups provide the family and the patient information to help them cope up with the pressures and challenges of the disorder.
    • Patient education or self management programs may help the patients and their families understand their current situations and help them control their lives.
    • The patient is also entitled for some benefits if their daily activities are affected.

Prognosis for Systemic Sclerosis

The disease is either moderate or mild for those with systemic sclerosis. For few patients, the disease can be life threatening. The prognosis depends largely on the type of systemic sclerosis the patient has and how fast the condition progresses. Specialists are enlisted to check the patient's prognosis with detail.
Patients who have the cutaneous systemic sclerosis will have a milder form and it will progress rather slowly. To those who have the diffuse cutaneous type, it will have a faster onset period and the complications are initially worst but it will stabilize after some time. The condition for some patients would even improve.

No cure has been found yet but many researches are being undertaken to help the patients with systemic sclerosis.

Important notice