World Scleroderma Foundation Preliminary Recommendations for Systemic Sclerosis Management During the COVID-19 Pandemic

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The World Scleroderma Foundation developed preliminary guidance based on clinical questions on the SARS-CoV-2 infection for systemic sclerosis management during COVID-19.

The World Scleroderma Foundation (WSF) has developed preliminary guidance based on practical clinical questions regarding severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection for the management of patients with systemic sclerosis (SSc) during the coronavirus disease 2019 (COVID-19) pandemic. The full report was published in Annals of the Rheumatic Diseases.

The presence of interstitial lung disease (ILD) and widespread use of immunosuppressive treatment may increase risk for a severe disease course and mortality in patients with SSc who may be infected with SARS-CoV-2. Therefore, the WSF released preliminary guidance developed by rheumatology, virology, and clinical immunology experts globally to address the main practical clinical questions regarding the COVID-19 pandemic.

Recommendations for SSc Management During COVID-19 Based on Clinical Questions

  • To prevent infection from SARS-CoV-2, patients with severe SSc-ILD should be recommended to follow the guidance of government and health authorities of their respective countries.
  • Patients should continue to receive immunosuppression to avoid SSc relapses; any drug withdrawal should be discussed and decisions made on a case-by-case basis. However, if patients or household members develop COVID-19, immunosuppression should be put on hold.
  • Patients with comorbidities such as diabetes, systemic hypertension, cardiovascular disease, and other chronic lung disease should be closely followed up, even in the early phases of the disease. Although the threshold to recommend hospitalization should be low, signs, symptoms, and baseline investigations should guide the recommendation for hospitalization.
  • Currently, testing the whole SSc population for COVID-19 is not advised. Patients at high-risk should be recommended to adhere to testing guidance from local and national authorities.
  • If patients with SSc show signs and symptoms of COVID-19, a diagnostic test should be performed, and quarantine be recommended while awaiting test results. Clinicians must closely follow-up with patients during this time, either digitally or by telephone. In situations of rapidly worsening conditions, including dyspnea and hypocapnic hypoxia, patients should be hospitalized and investigated with nasal swab retesting, serology and/or bronchoalveolar lavage.
  • With regard to stopping immunosuppressive treatment in patients who are tested as COVID-19-positive, the degree of SSc-ILD and the risk for disease/progressive damage or flare vs a potential higher risk for COVID-ILD evolution should be considered. Because of limited published evidence, future studies on the effects of immunosuppression in severe COVID-19 need to be closely monitored.
  • The recommendations indicated that no change in therapy, including angiotensin receptor blockers and angiotensin-converting enzyme inhibitors, was necessary for vascular and renal involvement in patients who are tested as COVID-19-positive.
  • Currently, no well-controlled, well-done trials are available for the prophylactic use of chloroquine, hydroxychloroquine (HCQ), or other adjunctive therapy in patients with SSc, therefore following international preventive measures, such as wearing masks and hand hygiene, is crucial and should be recommended.
  • Antiviral therapy or tocilizumab may be considered as a rescue treatment among patients with bilateral and severe COVID-19 pneumonia. Although evidence is lacking, antimalarials may be administered to patients with SSc-COVID-19: chloroquine 500 mg twice a day for 20 days or HCQ 200 mg twice a day from 5 to 20 days. The recommendations suggest preventive anticoagulation for infected, hospitalized patients with SSc.
  • Patients should be encouraged to limit their visits to the hospital/clinic until a more suitable time, although this may be determined on individual case basis. Telemedicine consultations have also been advised.

“This preliminary advice is aligned with other national and international recommendations, adapted for patients [with SSc],” the authors of the recommendations noted.

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Matucci-Cerinic M, Bruni C, Allanore Y, et al. Systemic sclerosis and the COVID-19 pandemic: World Scleroderma Foundation preliminary advice for patient management [published online April 29, 2020]. Ann Rheum Dis. doi:10.1136/annrheumdis-2020-217407