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The disease you did not know: this is what you must know about scleroderma

Many patients with scleroderma may have lung disease that can lead to deterioration in their condition and endanger their lives.

Now, thanks to a new and effective treatment, and with the help of a quick and accurate diagnosis, their chances of a better medical response increase.

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• Lungs

Prof. Uri Elkayam, in collaboration with the Association for Patient Rights

Thursday, 27 May 2021, 11:00 Updated: 11:13

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It is sometimes manifested by a change in the color of the hands.

Handheld (Photo: ShutterStock)

Scleroderma (Systemic Sclerosis) is a chronic autoimmune disease in which collagen protein accumulates in the connective tissue in the body.

About 60 to 80 percent of patients with scleroderma have health damage of varying degrees of severity.

Healthy connective tissue involvement is characterized by shortness of breath and exertional fatigue to the point of shortness of breath at rest.

(Of the connective tissue diseases, scleroderma is characterized by the highest incidence of involvement of interstitial lung diseases).

It is a serious disease that can worsen the patient's condition and endanger his life, and therefore requires a quick and accurate diagnosis, follow-up and proper treatment.

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In the first stage, the disease must be diagnosed. It has unique symptoms that appear in most cases around the age of 40, such as the Renault phenomenon which manifests itself in changes in the color of the hands on exposure to cold, joint and muscle pain, changes in skin texture, general weakness, sometimes heartburn and difficulty swallowing and feeling sick. As noted above, pulmonary involvement is common and manifests itself in coughing and shortness of breath. This involvement may be latent at first but later, in a significant percentage of patients, the lung disease may deteriorate and in extreme cases lead to respiratory failure to the point of needing a lung transplant.

Therefore, along with the initial diagnosis of scleroderma disease, patients should be referred for CT and lung function tests, even in cases where no signs of lung disease have appeared. It is important to do this as early as possible to get a good picture of possible pulmonary involvement. This is the best opportunity to identify the disease in its early stages, delay its progression and provide the best treatment to the patient.

The treatment plan for scleroderma patients who have started to develop lung disease has, until recently, been based mainly on treatments that focused on the autoimmune part of the disease, whether it is first-generation treatment that appeared 20 years ago, based on light chemotherapy or more advanced treatments.

All of these treatments have caused some stabilization of the disease, but in fact their benefit over time is limited.

Moreover, their effect on the course of the disease in the long run has not been unequivocally proven.

But these were the only treatments available.

Lung disease may worsen.

Doctor looks at lung scan (Photo: ShutterStock)

The change began in recent years when studies were presented that showed how treatment that works in the mechanism that attacks the pathological process itself manages to cause a 44 percent slowdown in the rate of deterioration of lung function, a significant slowdown, no doubt. Since the study was published, treatment has been added to the health basket in Israel, thus adding a significant therapeutic measure for many patients who need it. Now that we have treatment that has shown good results in slowing down the disease, the importance of early detection of the disease, monitoring and providing an accurate and quick response to any possible exacerbation is increasing.

The past year, along with the corona epidemic, has been a particular challenge for patients with pulmonary involvement in scleroderma - both due to lung disease and due to immunosuppressive treatments that may be risk factors for corona complications. Most patients have succeeded this year thanks to the patients' high awareness of social distance, thanks to the continuous, sometimes distant, contact with the doctors and doctors and thanks to the high response to the vaccination campaign.

It is important to emphasize that the treatment of chronic diseases during corona is essential and that along with adherence to the guidelines, it is of great importance to carry out all the recommended follow-up measures in order to enable optimal treatment.

The author is Prof. Uri Elkayam, Director of the Rheumatology Institute, Tel Aviv Medical Center

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