Objective To examine whether early therapeutic intervention, compared with delayed intervention, is beneficial for patients with early systemic sclerosis (SSc). Methods This is a single-center, retrospective cohort study of SSc patients who received cyclophosphamide, mycophenolate mofetil, methotrexate, or tocilizumab for diffuse cutaneous SSc (dcSSc) or interstitial lung disease (ILD) within 6 years after disease onset. The patients were divided into early and delayed intervention groups based on the disease duration of ≤ 18 and >18 months at treatment introduction, respectively. Clinical worsening was defined as the development of any original or revised ACR Composite Response Index in Systemic Sclerosis (CRISS) step 1 event or progressive fibrosing ILD (PF-ILD). Results There was no difference in baseline characteristics between the early (n = 25) and delayed (n = 21) intervention groups except forced vital capacity (FVC), which was better in the early vs delayed intervention groups. The early intervention group less frequently had stable pulmonary function over one year than did the late intervention group (odds ratio 0.087, 95% confidence interval 0.0079–0.51, p = 0.003). The active disease was significantly decreased from 79% to 42% in the early intervention group (p = 0.007), but the change in the delayed intervention group was not statistically significant (68% to 42%, p = 0.11). Cumulative rates free from clinical worsening events defined by revised ACR-CRISS and PF-ILD were significantly higher in the early vs delayed intervention groups (p = 0.03 and 0.003, respectively). Conclusion A therapeutic “window of opportunity” might exist in SSc patients.
Objectives To evaluate the effectiveness of rituximab (RTX) in patients with systemic sclerosis (SSc). Methods PubMed was searched for articles, published through March 31, 2022, on any controlled studies using RTX in the treatment of SSc. Of 85 identified articles, nine were selected by title/abstract screening and full text examination. All nine articles reported outcomes of forced vital capacity (%FVC), and seven reported those of modified Rodnan skin scores (mRSS). Results Among the seven controlled studies evaluating skin lesions in patients with SSc, four showed a significant improvement of mRSS by RTX when compared with a control group, whereas three show no significant effect. Among the nine controlled studies evaluating lung lesions, five showed a significant improvement of %FVC compared with a control group, whereas four showed no significant effect. Conclusions RTX may be effective in the treatment of skin and lung lesions in patients with SSc. The profiles of SSc patients for whom RTX is indicated were unclear, although patients with diffuse cutaneous SSc and those positive for anti-topoisomerase I antibody were considered potential targets. Additional studies are needed to assess the long-term effectiveness of RTX in the treatment of patients with SSc.
BackgroundThe second COVID-19 vaccination in autoimmune disease (COVAD-2) study [1] is an international, multicentre, self-reported e-survey designed to evaluate several facets covering COVID-19 infection and vaccination as well as validated patient-reported outcome measures (PROMs) in a variety of autoimmune diseases (AIDs), including systemic sclerosis (SSc). Detailed assessment of the health-related quality of life (HRQOL) and its drivers in patients with SSc is lacking.ObjectivesTo assess physical and mental health in a global cohort of SSc patients in comparison with non-SSc autoimmune inflammatory rheumatic diseases (AIRDs), non-rheumatic AIDs (NRAIDs), and those without AIDs (controls) using Patient-Reported Outcome Measurement Information System (PROMIS) global health data from the COVAD-2 survey.MethodsThe COVAD-2 database was used to extract demographics, AID diagnosis, comorbidities, disease activity, current therapies, and PROMs. PROMIS global physical health (GPH), global mental health (GMH) scores, PROMIS physical function short form-10a (PROMIS PF-10a), pain visual analogue scale (VAS), and PROMIS Fatigue-4a scores were compared between SSc, non-SSc AIRDs, NRAIDs, and controls. Outcomes were also compared between diffuse cutaneous SSc (dcSSc) vs limited cutaneous SSc (lcSSc). Multivariable regression analysis was performed to identify factors influencing GPH and GMH scores in SSc.ResultsA total of 10,502 complete responses from 276 SSc, 6006 non-SSc AIRDs, 545 NRAIDs, and 3675 controls as of May 2022 were included in the analysis. Respondents with SSc were older [SScvs.non-SSc AIRDsvs. NRAIDsvs. controls: 55 (14)vs.51 (15)vs. 45 (14)vs. 40 (14) years old, mean (SD), p < 0.001]. Among patients with SSc, 129 (47%) had dcSSc and 147 (53%) had lcSSc. SSc patients reported a significantly higher prevalence of ILD [SScvs.non-SSc AIRDsvs. NRAIDsvs. controls: 30.4%vs.5.5%vs. 1.5%vs. 0.2%, p < 0.001], and treatment with MMF [SScvs.non-SSc AIRDsvs. NRAIDsvs. controls: 26.4%vs.9.5%vs. 1.1%vs. 0%, p < 0.001].Patients with SSc had lower GPH and PROMIS PF-10a scores [SScvs.non-SSc AIRDsvs. NRAIDsvs. controls: 13 (11–15)vs.13 (11–15)vs. 15 (13–17)vs. 17 (15–18), median (IQR), p < 0.001; 39 (33–46)vs.39 (32–45)vs. 47 (40–50)vs. 49 (45–50), p < 0.001, respectively] and higher Pain VAS and PROMIS Fatigue-4a scores compared to those with NRAIDs or controls [SScvs.non-SSc AIRDsvs. NRAIDsvs. controls: 3 (2–5)vs.3 (1–6)vs. 2 (0–4)vs. 0 (0–2), p < 0.001; 11 (8–14)vs.11 (8–14)vs. 9 (7–13)vs. 7 (4–10), p < 0.001, respectively]. Patients with AIDs including SSc had lower GMH scores compared to controls [SScvs.non-SSc AIRDsvs. NRAIDsvs. controls: 12.5 (10–15)vs.13 (10–15)vs. 13 (11–16)vs. 15 (13–17), p < 0.001].Among SSc patients, GPH, GMH, and PROMIS PF-10a scores were lower in dcSSc compared to lcSSc [dcSScvs.lcSSc: 12 (10–14)vs.14 (11–15), p < 0.001; 12 (10-14) vs. 13 (10-15), p<0.001; 38 (30–43)vs.41 (34–47), p < 0.001, respectively]. Pain VAS and PROMIS Fatigue-4a scores were higher in dcSSc compared to lcSSc [4 (2–6)vs.3 (1–5), p < 0.001; 12 (8–15)vs.9 (8–13), p < 0.001, respectively].The independent factors for lower GPH scores in SSc were older age, Asian ethnicity, glucocorticoid use, and higher pain and fatigue scales, while mental health disorders and higher pain and fatigue scales were independently associated with lower GMH scores.ConclusionIn a global cohort, patient-reported physical and mental health were significantly worse in patients with SSc in comparison to those with non-SSc AIDs and without AIDs. Our findings support the critical need for more attention to patient’s subjective experiences including pain and fatigue to improve the HRQOL in patients with SSc.Reference[1]Fazal ZZ, Sen P, Joshi M, et al. COVAD survey 2 long-term outcomes: unmet need and protocol.Rheumatol Int. 2022; 42: 2151–58.Acknowledgements:NIL.Disclosure of InterestsKeina Yomono: None declared, Yuan Li: None declared, Vahed Maroufy: None declared, Naveen Ravichandran: None declared, Akira Yoshida: None declared, Kshitij Jagtap: None declared, Tsvetelina Velikova Speakers bureau: Pfizer and AstraZeneca, Parikshit Sen: None declared, Lorenzo Cavagna: None declared, Vishwesh Agarwal: None declared, Johannes Knitza: None declared, Ashima Makol: None declared, Dey Dzifa: None declared, Carlos Enrique Toro Gutierrez: None declared, Tulika Chatterjee: None declared, Aarat Patel: None declared, Rohit Aggarwal Consultant of: Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therepeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio, Grant/research support from: Bristol Myers-Squibb, Pfizer, Genentech, Octapharma, CSL Behring, Mallinckrodt, AstraZeneca, Corbus, Kezar, Abbvie, Janssen, Kyverna Alexion, Argenx, Q32, EMD-Serono, Boehringer Ingelheim, Roivant, Merck, Galapagos, Actigraph, Scipher, Horizon Therepeutics, Teva, Beigene, ANI Pharmaceuticals, Biogen, Nuvig, Capella Bioscience, and CabalettaBio, Latika Gupta: None declared, Masataka Kuwana Speakers bureau: Abbvie, Asahi-Kasei, Astellas, Boehringer-Ingelheim, Chugai, Eisai, MBL, Mochida, Nippon Shinyaku, Ono Pharmaceuticals, Tanabe-Mitsubishi, Consultant of: Astra Zeneka, Boehringer-Ingelheim, Chugai, Corbus, GSK, Horizon, Tanabe-Mitsubishi, Grant/research support from: Boehringer-Ingelheim, Vikas Agarwal: None declared.
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