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Scleroderma

Scleroderma is an autoimmune disease affecting the skin and other organs of the body, meaning that the body’s immune system is causing inflammation and other abnormalities in these tissues.

It often leads to skin tightening and thickening, and may affect the heart, lungs, kidneys, blood vessels or intestines

The word “scleroderma” comes from two Greek words: “sclera” meaning hard, and “derma”, meaning skin. Hardening of the skin is one of the most visible manifestations of the disease.

The main finding in scleroderma is thickening and tightening of the skin and inflammation and scarring of many body parts, leading to problems in the lungs, kidneys, heart, intestinal system and other areas.

There is still no cure for scleroderma, but effective treatments for some forms of the disease are available.

It is broadly classified into two- Local and systemic.

One of the earliest symptoms is Raynaud’s. This term refers to color changes (blue, white and red) that occur in fingers (and sometimes toes), often after exposure to cold temperatures. It occurs when blood flow to the hands and fingers is temporarily reduced.

Other symptoms include Skin thickening, swelling and tightening, joint pain, Calcium deposits in the skin or other areas. It can progress to involve the gastrointestinal tract causing heartburn, bloating and the chest causing shortness of breath and dry cough.

Scleroderma is relatively rare, most are women between the ages of 30 and 50.

Survival in patients with diffuse cutaneous disease has improved significantly; currently, the 5-year survival is estimated to be about 80%.

Five-year survival in patients with limited cutaneous disease is approximately 90%

Scleroderma causes significant physical distress, is disfiguring, and can decrease normal life expectancy

The most common first sign of scleroderma is Raynaud’s phenomenon, a clinical problem of cold and stress induced vasospasm of the digital arteries and cutaneous arterioles involved in body thermoregulation.

After the onset of Raynaud’s phenomenon, patients may be otherwise asymptomatic for years or they may rapidly develop other early symptoms and signs of disease activity such as fatigue, weight loss, musculoskeletal pain, gastrointestinal reflux disease (GERD), nailfold capillary changes, edema in the extremities or obvious skin thickening

Skin thickening is the most obvious physical finding to make a diagnosis of scleroderma

Facts on Raynaud’s

  1. Raynaud’s phenomenon, often just called Raynaud’s, is a condition where the small blood vessels of the fingers become narrow (constrict), most commonly when they are in a cold environment.
  2. It can also be triggered by stress or emotional changes.
  3. Typically, symptoms develop in fingers when you become cool – for example, in cold weather.
    • At first the fingers go white and cool. This happens because the small blood vessels in the fingers narrow (constrict).
    • The fingers then go a bluish colour (or even purple or black in severe cases). This happens because the oxygen is used up quickly from the blood in the narrowed blood vessels.
    • The fingers then go bright red. This happens because blood vessels open up again (dilate) and the blood flow returns. This may cause tingling, throbbing, numbness and pain (which can be severe in some cases).

4. Raynaud’s phenomenon may be present for many years before any other clinically significant symptoms or systemic manifestations occur.

5. About 1 in 20 people develop Raynaud’s phenomenon.

6.Up to 9 in 10 cases are primary Raynaud’s. Primary Raynaud’s usually first develops in teenagers and young adults, but it can develop at any age.

7. Secondary Raynaud’s can develop at any age when the underlying condition develops. Its associated with scleroderma (90%), lupus, rheumatoid arthritis amongst other connective tissue disease.

Features that may suggest secondary Raynaud’s include:

  • Onset of symptoms after 30 years of age.
  • Abrupt onset with rapid progression and worsening of symptoms.
  • Severe symptoms that may include an ulcer or gangrene of part of a finger or toe.
  • Symptoms that only affect one hand or foot, or the symptoms are not the same or as severe on both hands and feet.
  • Joint pains or arthritis.
  • Skin rashes.
  • Dry eyes or mouth.
  • Muscle weakness or pain.
  • Swallowing difficulties.
  • Mouth ulcers.
  • Previous work with vibrating tools

What can I do to reduce symptoms of Raynaud’s?

  1. Quit smoking as it worsens the symptoms
  2. Such medicines include beta-blockers, some anti-migraine medicines, decongestants and, very occasionally, the contraceptive pill.
  3. Caffeine (in tea, coffee, cola and in some painkillers) triggers symptoms in some people. Try cutting out caffeine for a few weeks to see if it helps. Amphetamines and cocaine may also be a trigger.
  4. Try to keep warm in cool weather or in cool environments
  5. Regular exercise is recommended by many experts. Exercise your hands and feet frequently to improve the circulation.
  6. When a bout of symptoms develops, warm the affected hands or feet as soon as possible. Soaking the hands or feet in warm running water is a good way to get warm (but take care that the water does not become too hot, or lose its heat and become cool).

 

What are the annual tests for scleroderma?

Additional issues that are an on going conversation are sexual well-being and mental health issues.

 

 

 

 

 

Source:

  1. Scleroderma UK
  2. John Hopkins Scleroderma Center
  3. UpToDate

 

 

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