Worse COVID-19 Outcomes Seen in Patients With Systemic Sclerosis


A retrospective study analyzed patients with systemic sclerosis to determine prevalence, severity, and mortality within this patient population.

Patients with systemic sclerosis interstitial lung disease (SSc-ILD) and forced vital capacity (FVC) <80% had a higher risk of moderate-to-severe COVID-19 infection and death, according to a study published in Seminars in Arthritis and Rheumatism.1

“Special attention should be given to patients with SSc, a rare heterogeneous autoimmune disease, with the highest mortality among autoimmune rheumatic diseases (ARDs),” investigators explained. “In particular, SSc-ILD is a frequent complication, affecting up to 70% of patients during the disease course.”

A multicenter, retrospective, observational study analyzed 1042 patients (87.6% female, mean age of 53.8 years) with SSc between March 2020 and June 2021 in São Paulo, Brazil. COVID-19 was determined by either a positive test or by “highly suspicious infection.” All cases were grouped into either mild or moderate-to-severe infection. Mild was defined as the absence of oxygen support and outpatient treatment, while moderate-to-severe was defined as needing supplemental oxygen and/or hospitalization.

Eligible patients were aged 18 years or older and met the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) 2013 classification criteria for SSc. Information collected included age, sex, disease subtype, disease duration, comorbidities, immunosuppressive therapy, and COVID-19 symptoms.

A total of 118 patients were diagnosed with COVID-19 during the study period, with 65.6% of patients diagnosed with SSc-ILD in the total group (46.3% of those with COVID-19). The prevalence of COVID-19 was comparable with the general population. The most common comorbidity was hypertension (24.6%), diabetes (9.3%), and obesity (9.3%). Most (73%) were currently using immunosuppressive drugs.

Most cases (66.1%, n = 78) were mild, while 33.9% (n = 40) had moderate-to-severe infection and 6 (5.1%) deaths were reported. Of those with moderate-to-severe disease, 18 were admitted to the intensive care unit and 8 needed mechanic ventilation. Lingering symptoms were observed in 40% of patients, with the most common symptoms being dyspnea and/or cough (17%) and musculoskeletal pain (12.5%).

Age, gender, corticosteroids, and comorbidities were not associated with worse outcomes. However, pulmonary arterial hypertension (OR 9.50, p=0.006), SSc-ILD (OR 3.90, p=0.007), FVC <80% (OR 2.90, p=0.01), cardiac involvement (OR 5.53, p=0.003), and use of rituximab (OR 3.92, p=0.039) increased the likelihood of worse outcomes.

Multivariate analysis indicated patients with SSc-ILD had a significantly increased chance of developing moderate-to-severe COVID-19 (OR 2.73, 95% CI 1.12-6.69, p=0.02).

The retrospective study design and small number of patients, severe SSc manifestations, and deaths limited the study. Few patients were currently being treated with rituximab, tocilizumab, and cyclophosphamide, which may have skewed results. Lastly, as the data was collected from centers in São Paulo, the results may not be generalizable to national outcomes for patients with SSc diagnosed with COVID-19.

“As vaccination is moving forward, the pandemic seems to be decreasing. Nonetheless, challenges continue to emerge, including the appearance of new SARS-CoV-2 variants, COVID-19 sequelae, the global emotional distress caused by the pandemic, and the disparities on vaccination coverage among the world population,” investigators concluded. “Therefore, it is important to be aware of specific populations with a higher risk for worse outcome, in order to implement specific health policies to better manage these individuals.”


de Oliveira SM, Martins LVO, Lupino-Assad AP, et al. Severity and mortality of COVID-19 in patients with systemic sclerosis: a Brazilian multicenter study [published online ahead of print, 2022 Feb 25]. Semin Arthritis Rheum. 2022;55:151987. doi:10.1016/j.semarthrit.2022.151987

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