scleroderma and CREST syndrome
Causes and risk factors
Scleroderma affects adults more often. In women, the disease occurs 4-8 times more often than in men. In particular, women become ill at the perimenopausal age.
The cause is still unknown. In some patients, such syndromes are caused by exposure to certain toxins or bone marrow transplantation.
Which are the symptoms?
The most characteristic signs are thickening and induration of the skin.
Usually, the disease manifests itself on the hands, especially on the fingers (sclerodactyly), and then spreads to other areas. Ulceration of the joints of the fingers and fingertips is common.
On the affected areas of the skin, hair loss is observed, the skin becomes shiny, the subcutaneous layers are compacted, wrinkles gradually disappear and the skin color changes.
- telangiectasia (small vessels that become visible on the skin);
- arthritis and muscle weakness;
- difficulty swallowing food;
- delayed peristalsis of the gastrointestinal tract with digestive problems;
- dry eyes and mouth;
- Raynaud’s phenomenon;
- cardiopulmonary lesion;
- kidney damage.
How is it diagnosed?
General laboratory tests are often not very indicative, with antinuclear or anti-centromeric antibodies present.
How is it treated?
There is no radical treatment, but there are measures to combat various aspects of the disease:
- drug therapy: for inflammation and arthralgia, heartburn, Raynaud’s phenomenon and, possibly, for organ damage;
- physical therapy: to maintain and preserve muscle tone and joint function.
More recently, the effectiveness of intravenous administration of prostacyclin analogues has been demonstrated. In some isolated cases, an attempt was made to transplant autologous bone marrow with peripheral stem cells.
The 5-year survival rate from the time of diagnosis is between 80 and 85%. The most serious problems occur due to damage to internal organs, especially the heart, lungs and kidneys.