Stabilization of Microcirculation in Patients with Early Systemic Sclerosis with Diffuse Skin Involvement following Rituximab Treatment: An Open-label Study

Smith, Vanessa; Pizzorni, Carmen; Riccieri, Valeria; Decuman, Saskia; Brusselle, Guy; Pauw, Michel De; Deschepper, Ellen; Piette, Yves; Ruaro, Barbara; Sulli, Alberto; Vandecasteele, Els; Melsens, Karin; Keyser, Filip De; Cutolo, Maurizio

doi:10.3899/jrheum.151018

Cited by 42 publications

(42 citation statements)

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“…At this time, the capillary loss score was unchanged (1.05 at baseline and 1.06 at 12 months) and an MES score increase of just 6.3% occurred. This was similar to that achieved in the study of Smith et al [ 5 ] in patients with early SSc. At 24 months, the patients had 24.5% and 28.3% mean increases in capillary loss score and MES, respectively.…”

Section: Main Textsupporting

confidence: 92%

“…Microcirculation evaluated by nailfold capillaroscopy is correlated with the severity of organ involvement [ 2 , 3 ]. Stem cell transplantation has been demonstrated to improve and rituximab to stabilize microcirculation in patients with SSc in the early stages of the disease [ 4 , 5 ]. Rituximab is a monoclonal antibody that inhibits B lymphocyte proliferation and is effective in the treatment of several autoimmune diseases [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

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Effects of treatment with rituximab on microcirculation in patients with long-term systemic sclerosis

et al. 2018

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ObjectiveTo investigate the effect of rituximab on microcirculation in long-term SSc.ResultsFour patients with diffuse SSc over 3 years of disease received rituximab cycles of two 1-g infusions every 6 months for 2 years. Videocapillaroscopy was performed at baseline, 12 months, and 24 months and semi-quantitative scoring of videocapillaroscopy abnormalities was performed and the microangiopathy evolution score (MES: range 0–9) was calculated. The mean disease duration was 5 years (range 3–15). On videocapillaroscopy, giant capillaries and hemorrhages remained stable from baseline to 24 months. Capillary loss, abnormally-shaped capillaries, and MES stabilized at 12 months and increased by 24.5% and 28% at 24 months. Rituximab improves microcirculation in long-term SSc. Stabilization and reduced progression of microcirculation abnormalities were achieved at 12 and 24 months, respectively.Electronic supplementary materialThe online version of this article (10.1186/s13104-018-3994-1) contains supplementary material, which is available to authorized users.

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Section: Main Textsupporting

confidence: 92%

Section: Introductionmentioning

confidence: 99%

Effects of treatment with rituximab on microcirculation in patients with long-term systemic sclerosis

et al. 2018

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“…From March to September 2017, out of 14 239 patients recorded in the database, 254 were recruited because of rituximab use (37 centres): 71% of female, median age: 51 years, 64% of diffuse cutaneous form and 71% displayed lung fibrosis. Median extent of lung fibrosis on CT was 20% 10–29. Anti-Scl70 and ACA antibodies were detected in 53% and 11% of the patients, respectively.…”

Section: Resultsmentioning

confidence: 97%

“…Some previous small uncontrolled or unadjusted series have suggested some efficacy of rituximab on cutaneous involvement in SSc 5–13 19 29. However, evaluation of drug efficacy in SSc might be difficult because of spontaneous improvement in skin fibrosis during the first years, highlighting the need of control group.…”

Section: Discussionmentioning

confidence: 99%

“…Despite the fatal burden associated with this condition, treatment options for SSc remain limited 4. Preliminary case-reports and series have suggested that rituximab, a chimeric monoclonal antibody targeting B cells, could improve skin and lung fibrosis in SSc 5–19. However, these studies were mostly limited by small samples sizes, lack of control arms, single centre designs, discrepancies in study designs and short durations of follow-up.…”

Section: Introductionmentioning

confidence: 99%

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Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Elhaï

Boubaya

Distler³

et al. 2019

Annals of the Rheumatic Diseases

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167

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ObjectiveTo assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice.MethodsWe performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab.Results254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47–5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55–1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56–3.53], p<0.0001). Patients treated concomitantly with mycophenolate mofetil had a trend for better outcomes as compared with patients receiving rituximab alone (delta FVC: 5.22 [0.83–9.62]; p=0.019 as compared with controls vs 3 [0.66–5.35]; p=0.012).ConclusionRituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.

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Ultrasound evaluation of endothelial dysfunction in immunoglobulin-resistant children with acute Kawasaki disease

et al. 2022

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Purpose Clinically, some children with Kawasaki disease (KD) who do not respond to the rst application of gamma globulin therapy have a longer course of disease and a higher risk of coronary artery damage. The characteristics of vascular endothelial dysfunction in intravenous immune globulin (IVIG)-resistant patients during the acute phase are not well described. We aimed to determine whether indicators that detect the situation of the endothelium are useful parameters that can accurately re ect subclinical dysfunction in resistant patients and may assist in differentiating patients with KD at a higher risk of IVIG resistance.Methods Fifty IVIG-resistant KD children, 120 IVIG-responsive KD children, 70 febrile children with acute upper respiratory infection and 100 healthy controls were recruited, and indicators re ecting endothelial in ammation, including ow-mediated dilation (FMD), were measured. Receiver operating characteristic (ROC) curve analysis was utilized to determine the threshold values of these parameters associated with IVIG resistance. Multiple logistic regression analysis was performed to determine whether FMD was an independent predictor of IVIG-resistant patients.Results Compared with normal children, febrile children and IVIG-sensitive KD patients, IVIG-resistant patients had lower FMD (P<0.05) and higher carotid artery intima-media (CIMT) (P<0.05). Laboratory data demonstrated that the percentage of neutrophils (N %) and the levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) and serum procalcitonin (PCT) were signi cantly higher in the IVIG-resistant group than in the IVIG-responsive group, while the levels of albumin (ALB) and serum sodium were signi cantly lower (P< 0.05). PCT, Na+ and FMD exhibited AUCs of 0.727, 0.653 and 0.698, respectively, in predicting IVIG resistance in KD through ROC analysis. PCT > 1.69 ng/ml, Na+ <133.2 mmol/l and FMD <5.79% were independent predictors of IVIG resistance in KD (OR, 95% CI, P< 0.05).Conclusion More severe endothelial dysfunction, especially lower FMD, was present in IVIG-resistant patients than in IVIGresponsive patients. It is a helpful diagnostic tool that provides supportive criteria to detect KD patients at a higher risk of IVIG resistance when FMD<5.79% in children. What Is KnownChildren with Kawasaki disease have declined FMD and Stiffness index (SI) values, as well as raised carotid intima media thickness (CIMT) which are related to the severity of endothelial dysfunction in later stage after the onset.What is new 1. FMD is lower and CIMT is higher in KD patients with IVIG-resistance than those without, in the acute stage. 2. FMD and PCT have a negative relationship with each other, and FMD<5.79% reminds a chance of IVIG-resistance.

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Stabilization of Microcirculation in Patients with Early Systemic Sclerosis with Diffuse Skin Involvement following Rituximab Treatment: An Open-label Study

Cited by 42 publications

References 9 publications

Effects of treatment with rituximab on microcirculation in patients with long-term systemic sclerosis

Effects of treatment with rituximab on microcirculation in patients with long-term systemic sclerosis

Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Ultrasound evaluation of endothelial dysfunction in immunoglobulin-resistant children with acute Kawasaki disease

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