Emergency Live - Pre-Hospital Care, Ambulance Services, Fire Safety and Civil Protection Magazine

Literally, scleroderma means hard skin.

However, this definition only takes into account part of the disease’s manifestations.

More correctly, it is called systemic sclerosis because it can affect several parts of the body.

It is an autoimmune disease that occurs mainly in women.

RARE DISEASES? TO LEARN MORE VISIT THE UNIAMO – ITALIAN FEDERATION OF RARE DISEASES BOOTH AT EMERGENCY EXPO

The clinical history of the disease is extremely variable and not all those affected present the same symptoms.

Almost all, however, experience as their first symptom an altered sensitivity to cold that manifests itself in a change in skin colour, which turns white or blue as the ambient temperature decreases.

This symptom is the Raynaud’s phenomenon, which may precede the other manifestations of the disease by some time.

Hands initially affected by Raynaud’s phenomenon may swell, become hard, i.e. fibrotic.

In varying degrees, this loss of elasticity, due to an alteration in the tissues that make up the scaffolding of the skin and internal organs (the ‘connective tissue’) involves other body sites: the face, arms, legs and trunk.

Some people are then at risk of developing respiratory problems: inflammation and fibrosis of the lungs make these organs less elastic and functional. In addition, blood circulation between the heart and lung is made difficult with the development of a particular type of hypertension: pulmonary hypertension, which prevents the blood from being properly oxygenated.

Most people with systemic sclerosis may have a stiffening of the oesophagus with a tendency to acid reflux or formation of ulcers and cuts on the fingers of the hands.

We do not yet know the cause of the disease, but some of the phenomena that lead to the development of complications related to it are well known.

In genetically predisposed persons, one or more external factors act as a trigger for an uncontrolled reaction that leads to activation of the immune system.

This reaction culminates in circulatory and organ damage and fibrosis.

Systemic sclerosis predominantly affects the female sex, typically around the time of menopause (between the ages of 45 and 60), but cases at a younger age are not entirely uncommon.

Yes. The disease is defined as rare, i.e. no more than 5 out of 10,000 people are affected.

This allows us to estimate that 20-25,000 people in Italy are affected.

There are no lifestyles or habits that can prevent this disease.

Certainly, the earlier it is diagnosed, the better the course and the possibility of preventing or treating any complications early on.

As mentioned above, very often the first ‘alarm bell’ is Raynaud’s phenomenon.

If one notices that the fingers of the hands turn white or blue with the cold, it would be useful to perform a capillaroscopy, a simple non-invasive examination that allows early visualisation of the circulatory changes typical of the disease.

Blood tests for autoantibodies can then be performed.

In this way, it is possible to discover a disease in the preclinical phase, i.e. even years before the typical manifestations of the disease appear, and to set up a programme of monitoring and prevention of complications.

To date, there is no unambiguous therapy for systemic sclerosis, or a therapy that allows a definitive cure.

The main goal of treatment is to slow down its progression and treat complications.

Today, there are many drugs, and others are in development, that can treat the complications of the disease.

These drugs are all the more effective the earlier the problems of systemic sclerosis are recognised, before any damage becomes irreversible.

This is why early diagnosis and regular check-ups are important.

Emergency Live Even More…Live: Download The New Free App Of Your Newspaper For IOS And Android

Psoriasis, An Ageless Skin Disease

Relapsing-Remitting Multiple Sclerosis (RRMS) In Children, EU Approves Teriflunomide

ALS: New Genes Responsible For Amyotrophic Lateral Sclerosis Identified

Psoriatic Arthritis: What Is It?

Rehabilitation Therapies In The Treatment Of Systemic Sclerosis

Psoriasis: It Gets Worse In Winter, But It’s Not Just The Cold That’s To Blame

Exposure To Cold And Symptoms Of Raynaud’s Syndrome

Let's talk about ventilation: what are the differences between NIV, CPAP and BIBAP?

Cupping, the pain relief therapy applied to the skin

Recognising the different types of vomit according to colour

Nocturnal enuresis in children: when and why do children pee in bed?

AHA: Sleep joins revamped list of heart health essentials

TIPS - Transjugular porto-systemic shunt: what it is, how it works and when it is…

G7 Summit in Elmau, Germany: THW rescuers in the field with around 1,000…

USA: FDA Approves Skyrizi to Treat Crohn’s Disease

Solferino 2022, the torches of the Red Cross shone again: 4,000 volunteers in…

Covid in Italy: 'Spike in infections, +58.9% in a week'

Omicron 5, Prof. Pregliasco explains what to expect from the new Covid variant

ALF, Ambulance Leadership Forum next September

Helicopter rescue in the Lombardy: the essential characteristics of helicopters…

Fotokite at the service of firefighters and security: the drone system is in…

Thermal imaging always at your fingertips with FLIR Ignite Cloud Service

Fotokite flies at Interschutz: here is what you will find in Hall 26, Stand E42

Emergency Live is the only multilingual magazine dedicated to people involved in rescue and emergency. As such, it is the ideal medium in terms of speed and cost for trading companies to reach large numbers of target users; for example, all companies involved in some way in the equipping of specialised means of transport. From vehicle manufacturers to companies involved in equipping those vehicles, to any supplier of life- saving and rescue equipment and aids.

Emergency Live Piazzale Badalocchio 9/b, 43126 Parma (PR) – Italy Phone: +39 340 2246247 VAT Number: IT02277610347 Write us: go to the form mail

This website is not intended for the purpose of providing medical advice. All information, content, and material is for information and educational purposes and are not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. All the contents inside this website are addressed to EMS, Rescue and Medical professionals. All the information in the following pages are focused on the health sector, medical devices, pharmaceutical products or products inside these categories, and they request the use of a professional of the health sector.