Course and prognosis | Scleroderma

Course and prognosis

The course of the disease is difficult to predict and cannot be deduced from the constellation of symptoms. It can happen that unforeseen, very serious courses occur, which lead to death within months. However, morphea is not life-threatening.

Women generally have a better prognosis than men. In systemic scleroderma, the infestation of the internal organs is crucial. The limiting form generally has a good prognosis.

However, 10% of patients develop high blood pressure in the pulmonary circulation (pulmonary hypertension), which dramatically increases the mortality rate in this patient group. The diffuse form has a rather poor prognosis. If the kidney is also affected, after ten years only 30% of these patients are still alive, the lung is hardened (fibrosed) and about 50% survive the next 10 years. In patients without heart, lung and kidney involvement, the 10-year survival rate is 71%.

History

Descriptions of symptoms that today make one think of the diagnosis scleroderma can be found in the writings of Hippocrates (460-370 BC). However, the descriptions were rather imprecise.Carlo Curzio first precisely formulated the constellation of symptoms in Naples in 1753. For example, he described the hardness of the skin, the tightness around the mouth and the hardness around the neck.

In 1847, Elie Gintrac then coined the term “scleroderma“. He considered the disease to be a pure skin disease. Only William Osler recognized that the internal organs are also involved in the disease process.