Systemic scleroderma is a disease characterized by rapid growth of fibrous (connective) tissue that leads to scarring of skin and internal organs. Approximately one in 10,000 individuals is affected. It is more common in women and most often develops around age 30 to 50.
Also, can systemic scleroderma be mild?
There are several different types of scleroderma that can vary in severity. Some types are relatively mild and may eventually improve on their own, while others can lead to severe and life-threatening problems.
Moreover, how do you test for 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.
How is sine scleroderma diagnosed?
Diagnosing sine sclerosis
If sine sclerosis is suspected, patients may need to undergo more extensive blood tests, urine tests to assess kidney function, and chest X-rays, or CT scans of the lung.
Is scleroderma sine fatal?
It is the most fatal of all the rheumatologic diseases. Systemic scleroderma is very unpredictable although most cases can be classified into one of four different general patterns of disease (see Classification).
What can I expect from systemic scleroderma?
Scleroderma complications range from mild to severe and can affect the: Fingertips. In systemic sclerosis, Raynaud’s phenomenon can become so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores. In some cases, the tissue on the fingertips may die.
What can mimic scleroderma?
However, similar features of hard and thick skin can be seen in other conditions which are often referred to as “scleroderma mimics”. These mimics include eosinophilic fasciitis, nephrogenic systemic fibrosis, scleromyxedema, and scleredema among others.
What does a scleroderma flare feel like?
Common symptoms of scleroderma may include painful joints (arthralgia), morning stiffness, fatigue, and/or weight loss. The intermittent loss (triggered by cold temperatures) of blood supply to the fingers, toes, nose, and/or ears (Raynaud’s phenomenon) is an early and frequent complaint of people with scleroderma.
What happens if scleroderma is left untreated?
Scleroderma causes a restrictive ventilatory defect (small lungs) secondary to an inflammatory process in the lung alveoli (air sacs). This process, left untreated, leads to fibrosis of the lung tissue (a fibrosing alveolitis) that interferes with normal gas exchange from the air to the blood.
What is the difference between sclerosis and scleroderma?
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 life expectancy of someone with systemic 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.