“Systemic sclerosis is a rare systemic disease that affects internal organs, especially of the heart, lung, kidneys, the gastrointestinal tract as well as the skin. Precisely for this reason, the suffix ‘systemic’ tells us how demanding it is to consider the disease but above all how much knowledge is necessary to be able to manage a patient of such complexity. Early diagnosis is essential today because it allows us to treat patients even before the damage is done. Therefore, being able to identify a patient in the first phase of the disease, characterized by an inflammatory imprint, becomes very important to be able to manage it, treat it adequately and therefore prevent the evolution towards fibrosis which represents damage responsible for reducing the quality of life. of those who suffer from it “. Like this Marco Matucci Cerinicfull professor of Rheumatology at the University of Florence.
The rheumatologist – speaking on the sidelines of the press conference “Rare diseases: the value of multidisciplinarity for the early diagnosis of pulmonary fibrosis in people with systemic sclerosis”, held in Rome on the initiative of the Honorable Fabiola Bologna, rapporteur of the Unified Text for Rare Diseases – remember how systemic sclerosis or scleroderma “affects women more frequently, especially between the ages of 35 and 60, with a female-male ratio of 5-6 to 1. Men are few but when they fall ill they generally have a much more aggressive and evolutionary disease with a shorter life expectancy than women ”.
The clinical manifestations are, on the one hand, the expression of suffering and vascular remodeling and, on the other, the replacement of tissues with connective tissue, devoid of the normal characteristics of organ elasticity and function. In most patients, the first sign of the disease is Raynaud’s phenomenon that is, the clinical condition in which the extremities (hands, feet, sometimes even nose and ears) change color in response to the decrease in environmental temperature or to emotional stress.
“The hands first become white, then bluish and then red due to the return of the blood – explains the expert -. But in addition to Raynaud’s phenomenon, the disease manifests itself with other early signs, such as the presence of circulating auto-antibodies and microvascular alterations in the capillaries around the nails, which we can see through a specific examination, capillaroscopy “. Systemic sclerosis “then progressively evolves over time leading to interstitial lung disease, which reduces respiratory capacity, up to involvement of the heart muscle and the gastrointestinal tract. So it can be said that it is a highly complex disease that requires frequent monitoring and adequate therapy to maintain the patient’s quality of life ”, he concludes.
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