Introduction: The impact of COVID-19 pandemic represents a serious challenge for 'frail' patients' populations with inflammatory autoimmune systemic diseases such as systemic sclerosis (SSc). We investigated the prevalence and severity of COVID-19, as well the effects of COVID-19 vaccination campaign in a large series of SSc patients followed for the entire period (first 38 months) of pandemic. Patients and method: This prospective survey study included 1755 unselected SSc patients (186 M, 1,569F; mean age 58.7 ± 13.4SD years, mean disease duration 8.8 ± 7.3SD years) recruited in part by telephone survey at 37 referral centers from February 2020 to April 2023. The following parameters were carefully evaluated: i. demographic, clinical, serological, and therapeutical features; ii. prevalence and severity of COVID-19; and iii. safety, immunogenicity, and efficacy of COVID-19 vaccines. Results: The prevalence of COVID-19 recorded during the whole pandemic was significantly higher compared to Italian general population (47.3 % vs 43.3 %, p < 0.000), as well the COVID-19-related mortality (1.91 % vs 0.72 %, p < 0.001). As regards the putative prognostic factors of worse outcome, COVID-19 positive patients with SSc-related interstitial lung involvement showed significantly higher percentage of COVID-19-related hospitalization compared to those without (5.85 % vs 1.73 %; p < 0.0001), as well as of mortality rate (2.01 % vs 0.4 %; p = 0.002). Over half of patients (56.3 %) received the first two plus one booster dose of vaccine; while a fourth dose was administered to 35.6 %, and only few of them (1.99 %) had five or more doses of vaccine. Of note, an impaired seroconversion was recorded in 25.6 % of individuals after the first 2 doses of vaccine, and in 8.4 % of patients also after the booster dose. Furthermore, the absence of T-cell immunoreactivity was observed in 3/7 patients tested by QuantiFERON® SARSCoV-2 Starter Set (Qiagen). The efficacy of vaccines, evaluated by comparing the COVID-19-related death rate recorded during pre- and post-vaccination pandemic periods, revealed a quite stable outcome in SSc patients (death rate from 2.54 % to 1.76 %; p = ns), despite the significant drop of mortality observed in the Italian general population (from 2.95 % to 0.29 %; p < 0.0001). Conclusions: An increased COVID-19 prevalence and mortality rate was recorded in SSc patients; moreover, the efficacy of vaccines in term of improved outcomes was less evident in SSc compared to Italian general population. This discrepancy might be explained by concomitant adverse prognostic factors: increased rate of non-responders to vaccine in SSc series, low percentage of individuals with four or more doses of vaccine, ongoing immunomodulating treatments, disease-related interstitial lung disease, and/or reduced preventive measures in the second half of pandemic. A careful monitoring of response to COVID-19 vaccines together with adequate preventive/therapeutical strategies are highly recommendable in the near course of pandemic in this frail patients' population.
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 / Ferri, Clodoveo; Raimondo, Vincenzo; Giuggioli, Dilia; Gragnani, Laura; Lorini, Serena; Dagna, Lorenzo; Bosello, Silvia Laura; Foti, Rosario; Riccieri, Valeria; Guiducci, Serena; Cuomo, Giovanna; Tavoni, Antonio; De Angelis, Rossella; Cacciapaglia, Fabio; Zanatta, Elisabetta; Cozzi, Franco; Murdaca, Giuseppe; Cavazzana, Ilaria; Romeo, Nicoletta; Codullo, Veronica; Pellegrini, Roberta; Varcasia, Giuseppe; De Santis, Maria; Selmi, Carlo; Abignano, Giuseppina; Caminiti, Maurizio; L'Andolina, Massimo; Olivo, Domenico; Lubrano, Ennio; Spinella, Amelia; Lumetti, Federica; De Luca, Giacomo; Ruscitti, Piero; Urraro, Teresa; Visentini, Marcella; Bellando-Randone, Silvia; Visalli, Elisa; Testa, Davide; Sciascia, Gabriella; Masini, Francesco; Pellegrino, Greta; Saccon, Francesca; Balestri, Eugenia; Elia, Giusy; Ferrari, Silvia Martina; Tonutti, Antonio; Dall'Ara, Francesca; Pagano Mariano, Giuseppa; Pettiti, Giorgio; Zanframundo, Giovanni; Brittelli, Raffaele; Aiello, Vincenzo; Dal Bosco, Ylenia; Foti, Roberta; Di Cola, Ilenia; Scorpiniti, Daniela; Fusaro, Enrico; Ferrari, Tommaso; Gigliotti, Pietro; Campochiaro, Corrado; Francioso, Francesca; Iandoli, Carlo; Caira, Virginia; Zignego, Anna Linda; D'Angelo, Salvatore; Franceschini, Franco; Matucci-Cerinic, Marco; Giacomelli, Roberto; Doria, Andrea; Santini, Stefano Angelo; Fallahi, Poupak; Iannone, Florenzo; Antonelli, Alessandro. - In: JOURNAL OF TRANSLATIONAL AUTOIMMUNITY. - ISSN 2589-9090. - 7:(2023), p. 100212. [10.1016/j.jtauto.2023.100212]