What are the types of systemic sclerosis?

Three Types of Systemic Sclerosis (Scleroderma): Limited, Diffuse and Sine.

What are the different types of scleroderma?

There are two main types or classes of scleroderma: localized scleroderma, which affects only certain parts of the body and includes linear scleroderma and morphea, and systemic sclerosis, which affects the entire body.

What are the stages of systemic sclerosis?

Cutaneous involvement has 3 phases: (1) edematous, (2) indurative, and (3) atrophic. Skin becomes thickened and tight.

What is the difference between scleroderma and systemic sclerosis?

What’s the difference between scleroderma and systemic sclerosis? Scleroderma is seen only on the skin. However, when the fibrosis involves your other internal organs, this is considered systemic sclerosis.

What is the difference between diffuse and limited systemic sclerosis?

This individual has skin thickening above the elbows and knees and on the chest and would be classified as having systemic sclerosis with diffuse scleroderma. An individual with skin thickening restricted to the face, forearms, hands and fingers would be said to have systemic sclerosis with limited scleroderma.

What is another name for scleroderma?

Overview. Scleroderma (sklair-oh-DUR-muh), also known as systemic sclerosis, is a group of rare diseases that involve the hardening and tightening of the skin.

What tests confirm systemic sclerosis?

Systemic sclerosis is diagnosed based on the presence of various symptoms above and physical examination findings. The diagnosis can be confirmed by the presence of certain autoantibodies in the blood as well as radiographic studies. Particularly, the ANA, or the antinuclear antibody test, is positive, but not always.

What causes systemic sclerosis?

Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. It is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs. The fibrosis is caused by the body’s production of too much collagen, which normally strengthens and supports connective tissues.

What is the treatment for systemic sclerosis?

Treatments are used which suppress the body’s immune system (because it is the immune system which is overactive in systemic sclerosis). Examples of these treatments aresteroids, methotrexate, cyclophosphamide, azathioprine, and mycophenolate mofetil. The medicines that are used will depend on the individual situation.

What is the mildest form of scleroderma?

Localised scleroderma is the mildest form of the condition. It often affects children, but can occur at any age. This type just affects the skin, causing 1 or more hard patches to develop. Internal organs aren’t affected.

What is the life expectancy for someone with scleroderma?

People who have localized scleroderma may live an uninterrupted life with only minor symptom experiences and management. On the other hand, those diagnosed with an advanced and systemic version of the disease have a prognosis of anywhere from three to 15 years.

What is the life expectancy of a person with scleroderma?

Over 30 years, the mean age of death improved from 66.4 to 74.5 years (P < 0.001). Duration of disease improved from 12.1 to 22.9 years (P < 0.001).

What is the survival rate for scleroderma?

Because the presentation and prognosis of SSc are highly heterogeneous, studies showed a 10-year survival rate between 50 and 84% [4, 18,19,20,21]. Such mortality is still related to SSc in 27 to 72% of cases [3, 6, 8, 16,17,18, 21,22,23,24,25,26,27].

Can you live a full life with systemic sclerosis?

Every patient with systemic sclerosis is different, which makes predicting additional complications and life span nearly impossible. Some patients with systemic sclerosis never develop severe involvement of their internal organs and live relatively normal lives.

How fast does systemic scleroderma progress?

In our cohort, 56% of observed progression occurred within 5 years since the first non-Raynaud symptom. While progression in skin involvement occurred more frequently in early disease, the proportion of patients with lung, heart or GI progression was relatively stable over time.

Is systemic sclerosis serious?

Systemic sclerosis (SSc, scleroderma) is the most serious form of the disease. This form of scleroderma can occur at any age but is most common in women of childbearing age.

What is the newest treatment for scleroderma?

Two drugs are currently FDA approved to treat lung scarring, or pulmonary fibrosis, that occurs as a result of scleroderma: nintedanib and tocilizumab.

What is the most common cause of death in systemic diffuse scleroderma?

Death from systemic sclerosis was most frequently due to pulmonary hypertension, with fewer than expected deaths from renal or cardiac causes. Twenty-eight per cent of deaths were due to causes unrelated to systemic sclerosis, most commonly cancer and ischaemic heart disease, and in older patients.

Is systemic sclerosis painful?

Systemic sclerosis can cause the tissues around joints to stiffen, which can reduce the range of movement of joints. It can also cause pain and swelling around affected joints.

How do you stop scleroderma progression?

Lifestyle and home remedies
  1. Stay active. Exercise keeps your body flexible, improves circulation and relieves stiffness. …
  2. Protect your skin. Take good care of dry or stiff skin by using lotion and sunscreen regularly. …
  3. Don’t smoke. …
  4. Manage heartburn. …
  5. Protect yourself from the cold.

What foods should be avoided with scleroderma?

Avoid foods that may aggra- vate symptoms such as citrus fruits, tomato products, greasy fried foods, coffee, garlic, onions, peppermint, gas-producing foods (such as raw peppers, beans, broccoli or raw onions), spicy foods, carbonated beverages and alcohol.