Prevalence of COVID-19 was greater in SSc patients in Italy

Vaccines also less effective in scleroderma patients, pandemic study finds

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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In Italy, the prevalence of COVID-19 was greater, with the virus resulting in a higher death rate, among people with systemic sclerosis (SSc) than in the general population, according to a study spanning the pandemic years.

The researchers also observed that while most SSc patients received three or more doses of a vaccine against COVID-19, some failed to mount an adequate immune response, meaning they may not be protected against infection.

In the event of a new pandemic, “a careful monitoring of response to COVID-19 vaccines together with adequate preventive/therapeutical strategies are highly recommendable,” the team wrote.

The study, “Impact of COVID-19 and vaccination campaign on 1,755 systemic sclerosis patients during first three years of pandemic. Possible risks for individuals with impaired immunoreactivity to vaccine, ongoing immunomodulating treatments, and disease-related lung involvement during the next pandemic phase,” was published in the Journal of Translational Autoimmunity.

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More deaths seen with greater prevalence of COVID-19 in SSc

Systemic scleroderma, also called systemic sclerosis, is an autoimmune disease in which thickened scar tissue builds up and replaces normal tissue. This scar tissue accumulates in the skin and several internal organs, such as the heart, kidney, lungs, and gastrointestinal tract.

Treatment for the rare disease often involves immunosuppressants, a type of medications that decrease the intensity of the body’s immune response. It’s known that some people who have a weakened immune system are more likely to get COVID-19 or be sick for longer.

To understand how common it was for people with SSc to get COVID-19, and whether vaccination protected against infection, a team of researchers from across Italy drew on data from 1,755 people with the disease. The patients comprised 1,569 women and 186 men, all of whom were followed remotely during the pandemic.

Nearly two-thirds (64.9%) had a diagnosis of limited scleroderma, a subtype of SSc, while the remaining one-third (35.1%) had diffuse scleroderma, which is usually more severe than the limited form. The mean age of the patients was 58.7, and their mean disease duration was 8.8 years.

Over the period spanning February 2020 to April 2023, COVID-19 was slightly but significantly more common among people with SSc than among the general Italian population (47.3% vs. 43.3%). This finding also was true for death from COVID-19 (1.91% vs. 0.72%).

“Scleroderma patients showed higher COVID-19 prevalence and mortality rate than [the] general population,” the researchers wrote. Prevalence is a measure of the total number of people in a group who have a certain disease during a given period of time.

Among people with SSc, those with interstitial lung disease (ILD) — a group of lung disorders marked by progressive scarring of lung tissue — were hospitalized significantly more often due to COVID-19 than were those without this complication (5.85% vs. 1.73%).

Death from COVID-19 also was approximately twice as common among people with SSc-related ILD (2% vs. 0.4%). Interstitial lung disease is a complication of SSc that occurs when lung tissue becomes scarred and inflamed, making it difficult to breathe and get enough oxygen from the lungs into the bloodstream.

Overall, having pre-existing ILD was linked with the “worst COVID-19 outcomes, namely increased percentages of both hospitalized and deceased patients,” the researchers wrote.

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Periodic checks of patients’ immune status urged to avoid symptoms

COVID-19 vaccines became more common starting in early 2021.

At that time, more than half (56.3%) of the individuals in the study had received two doses of a vaccine against COVID-19, plus one booster dose. A fourth dose was given to slight more than one-third of the patients in the study (35.6%). A small proportion (1.99%) had five or more doses of a vaccine.

Immune response was evaluated by testing the blood of 495 people with SSc for the presence of antibodies against SARS-CoV-2, the virus that causes COVID-19. About 1 in 4 patients (25.6%) failed to mount an effective immune response after two doses; even after a booster dose, 8.4% did not have antibodies against SARS-CoV-2.

These observations led the researchers to argue that people with active SSc may not be well-protected against COVID-19. This may be due to the immunosuppressants they take, which may affect how well vaccines work, or these patients may need additional booster doses.

“Therefore, a periodic check of [the] patients’ immune status is recommended in order to avoid a dangerous appearance of COVID-19 manifestations in a frequently frail patient because of multi-organ scleroderma involvement,” the researchers wrote.

In patients with no or low disease activity and impaired immune response to vaccination, “it is possible to discontinue the ongoing therapy and proceed with vaccination or booster dose administration,” the researchers added.

The introduction of anti-COVID-19 vaccines since the early 2021 did not affect significantly the COVID-19-related mortality in the SSc patients, despite the improved COVID-19 outcomes observed in the Italian general population in the same period of pandemic.

The efficacy of vaccines was evaluated by comparing the proportion of COVID-19-related deaths before and after vaccination. In the general population, this proportion dropped significantly from 2.95% to 0.29%, whereas in people with SSc, there was a non-significant drop of 0.78% (from 2.54% to 1.76%).

“The introduction of anti-COVID-19 vaccines since the early 2021 did not affect significantly the COVID-19-related mortality in the SSc patients, despite the improved COVID-19 outcomes observed in the Italian general population in the same period of pandemic,” the researchers wrote.

This could be due to a lack of efficacy of vaccines, the use of immunosuppressants, or the presence of lung complications. Moreover, fewer preventive measures were taken in the second half of the pandemic, the researchers noted.

“Therefore, a careful monitoring of individual patients should take into account the SSc activity and progression, the ongoing therapies, and the immune status towards SARS-CoV2 infection,” they wrote.

“All together these elements can usefully direct the disease treatment strategies and the timing of vaccine booster doses, as well as the correct management of a possible emergence of COVID-19 manifestations,” the researchers concluded.