Experts Issue Guidance for Care of Scleroderma Patients During COVID-19

With the World Scleroderma Foundation (WSF)’s support, experts worldwide answered questions in the form of  recommendations for scleroderma patients amid the COVID-19 outbreak, including the importance of maintaining current medications and discussing any changes with their doctors.

Their guidelines, “Systemic sclerosis and the COVID-19 pandemic: World Scleroderma Foundation preliminary advice for patient management,” were published in the Annals of the Rheumatic Diseases. 

COVID-19 is caused by the new SARS-CoV-2 virus. While information on both virus and resulting disease is limited, what is known to date suggest that people with scleroderma can be at greater risk for more severe complications than the general population.

Scleroderma is often associated with interstitial lung disease (ILD), which could exacerbate COVID-19 symptoms. Many scleroderma patients also use therapies that limit the activity of the immune system (immunosuppressants), which could make them more susceptible to viral infections.

A panel of experts with specialties that include rheumatology, virology, and clinical immunology met online to address the care of people with scleroderma during the pandemic. Their guidelines were based around common questions asked by people with the disease and their healthcare providers, and drew on information known about COVID-19 up to April 14.

“This preliminary advice is aligned with other national and international recommendations, adapted for SSc [systemic sclerosis, or scleroderma] patients,” they wrote.

The guidelines first emphasize the importance of preventive measures, such as proper social distancing, regular hand washing, and wearing face masks.

People with severe ILD should follow the advice of government and health authorities. In general, scleroderma patients should limit going to the hospital, but balance need against the risk of a disease flare and decide on a case-by-case basis. Online or telephone contact is preferred to in-person consultations.

Currently, no evidence supports a particular medication reducing a person’s COVID-19 risk. As such, the guidelines do not recommend use of chloroquine or hydroxchloroquine, both malaria treatments, for such purpose.

Although immunosuppressants can increase infection risk, these experts do not recommended that scleroderma patients stop taking their medications, as doing so could worsen disease symptoms. If a patient or family member develops COVID-19, immunosuppression it “may be advised” that immunosuppressant use be put on hold. Evidence supporting precise advice regarding continuing or discontinuing their use is still insufficient, experts said.

“The degree of SSc-ILD and the risk of disease/progressive damage or flare versus a potential higher risk of COVID-ILD evolution should be considered,” they wrote, citing one published case of a woman with systemic scleroderma and ILD who did postpone treatment until a mild coronavirus infection passed. “Further publications on the effects of immunosuppression in severe COVID-19 need to be closely monitored.”

In turn, it is not recommended that patients stop taking medications for kidney and vascular disease, such as angiotensin converting enzyme inhibitors.

Also, if they develop COVID-19 symptoms — such as fever, cough, or loss of the sense of smell — people with scleroderma should undergo a COVID-19 test. While awaiting results, the patient and those they have been in contact with should quarantine. If symptoms rapidly worsen, the patient should be hospitalized.

Some scleroderma-associated complications, such as diabetes, high blood pressure and cardiovascular disease, may increase the risk of poor outcomes from COVID-19. “Consequently, patients with these comorbidities should be closely followed, even in the early phase of the disease,” the experts wrote.

Regarding treatments for COVID-19 itself, data are lacking to say with certainty that any given option is effective. Yet, the guidelines suggest that antiviral therapies or antimalarial medications (e.g., chloroquine) may help, while therapies to prevent coagulation are advised in hospitalized scleroderma patients with COVID-19.

The WSF and the European Scleroderma Trial And Research (EUSTAR) group will launch a database dedicated to scleroderma-COVID-19 patients, the panel noted.

“In conclusion, SSc patients are a great challenge for the physician to achieve an effective protective strategy or, when infected, to optimise a real-time treatment as suggested by the rapidly evolving guidelines,” the experts concluded.