Table of Contents
It is celebrated on June 29th World Scleroderma Day. It is a rare disease, which can be better tackled if recognized early and treated accordingly.
Unfortunately, however, some problems are sometimes underestimated, also because the disease can present itself in different forms and is not easy to identify. However, there is one certainty: scleroderma speaks to women: it mainly affects women with a ratio of 10 to 1 compared to males. To remember it are the experts.
Because we need to increase attention to the topic
“It is important to know about scleroderma because it is a rare disease of which very little is said, despite being highly disabling – points out Roberto Caporali, Director of the Department of Rheumatology of the ASST Gaetano Pini-CTO of Milan, who dedicates a special event to the topic. Yet, if the diagnosis is early, it is possible to keep the symptoms under control and guarantee the patient a good quality of life. If specialists have to do their part, through the clinical analysis of patients, it is however essential that people know what are the first signs that can indicate the onset of the disease and when it is necessary to consult a specialist “.
And then, let’s try to identify some “signal” that can help suspect the diagnosis, so we need to talk to the doctor. Scleroderma is chronic inflammatory disease with autoimmune pathogenesis that affects the skin, microcirculation and sometimes some internal organs (lung, heart, kidney, gastrointestinal tract).
Generally two forms are recognized: one that mainly affects the circulatory system and the other in which the skin is most affected and the involvement of internal organs is frequent, such as the lungs, the heart or the gastrointestinal system and the joints. Where present, skin fibrosis, arthritis and skin ulcers cause a reduction in the ability to perform some ordinary activities.
In other patients, if present, cardio-pulmonary involvement may result in decreased respiratory capacity and reduced resistance to exertion. “Finally, in the mildest forms, the main ailment consists solely of Raynaud’s phenomenon, which can be managed with adequate specific therapy – explains Nicoletta Del Papa, rheumatologist in charge of the Scleroderma Clinic of the ASST Gaetano Pini-CTO”.
An examination helps in the diagnosis
Raynaud’s Phenomenon it is a symptom that is found in about 95% of patients: it consists of a vasospastic disorder triggered by exposure to low temperatures and / or stress and manifests itself with pallor followed by cyanosis (purplish color of the skin).
“Due to the phenomenon – adds Del Papa – the patient perceives numbness, tingling and the temporary loss of sensitivity. It is more localized in the hands and feet, sometimes also in the nose and ears. In the beginning, skin thickening is more common in the hands and fingers. Later, especially in the diffuse form, it also extends to other areas of the body “.
And then? So if you have manifestations of this type, the doctor can proceed with a simple and non-invasive examination. It is called capillaroscopy: it allows to analyze the microcirculation of the fingers of the hands. To have a certain diagnosis it is then necessary to undergo specific blood tests, which must be in line with the patient’s clinical evaluation.
As mentioned, however, we must remember a message: although scleroderma can be highly disabling, especially in the most serious cases, with the right therapy it is possible to keep the symptoms under control. The important thing, as often happens in medicine, is to know.
