Rituximab or cyclosporin in refractory immune thrombocytopenia secondary to connective tissue diseases: a real-world observational retrospective study

Sun, Fangfang; Chen, Jie; Wu, Wanlong; Geng, Shikai; Xu, Wenwen; Sun, Shuhui; Chen, Zhiwei; Gu, Liyang; Wang, Xiaodong; Li, Ting; Ye, Shuang

doi:10.1007/s10067-020-05152-x

Cited by 11 publications

(9 citation statements)

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“…Furthermore, the prevalence of aPL (16.5%) seemed to be numerically lower than previous reports in Chinese SLE cohorts including ours (20%-30%). [31][32][33][34][35] The meaning and underlying cause is unknown. It is noteworthy that discontinuation or reduction of HCQ was associated with lupus flares.…”

Section: Discussionmentioning

confidence: 99%

Risk factors of disease flares in a Chinese lupus cohort with low-grade disease activity

et al. 2022

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ObjectiveRecurrent disease flare is one of the key problems in lupus patients. A Chinese Flare-Prevention Lupus Initiative Cohort (FLIC) was established. Risk factors of disease flare were evaluated accordingly.MethodsPatients with low-grade disease activity (the Safety of Estrogens in Lupus Erythematosus National Assessment–SLE Disease Activity Index (SELENA-SLEDAI) =≤6, daily prednisone ≤20 mg, no British Isles Lupus Assessment Group A or no more than one B organ domain score) from January 2014 to August 2020 were included in the FLIC. Disease flares were defined by the modified SELENA-SLEDAI Flare Index. Low disease activity status (LDAS) and remission were also assessed. The cumulative flare rate was estimated by an event per 100 person-years analysis. Cox proportional hazards models were performed to identify risk factors of subsequent disease flares after adjusting clinical confounders. Survival was assessed with the Kaplan-Meier method.Results448 eligible patients with low-grade disease activity were included in FLIC. During a mean follow-up of 30.4 months, 170 patients flared. The cumulative lupus flare rate was 22.2 events per 100 patient-years. Compared with patients without flare, those with lupus flares were taking more prednisone, had higher disease activity index and with less patients attained LDAS/remission at baseline. They also had higher rates of antiphospholipid antibodies (aPLs) and antiribosomal P antibody. Cox regression analysis confirmed that attainment of either LDAS or remission at baseline were independent protective factors against subsequent disease flare (LDAS but not in remission: HR 0.58, 95% CI 0.38~0.88; remission: HR 0.46, 95% CI 0.30~0.69), while aPL was a risk factor of lupus flares (HR 1.95, 95% CI 1.36~2.78). Kaplan-Meier curves indicated that attaining LDAS or remission and absence of aPL at baseline had the least flare risk.ConclusionsIn our real-world cohort study, not attaining LDAS or remission at baseline and aPL positivity was associated with higher risk of disease flares in patients with low-grade SLE.

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Section: Discussionmentioning

confidence: 99%

Risk factors of disease flares in a Chinese lupus cohort with low-grade disease activity

et al. 2022

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“…A total of 23 reports have been published involving the use of CyA in AIC for a total of 441 patients ( Table 3 ). They were mainly pediatric studies (60%) and included 10 case reports/series, 9 – 18 8 clinical trials, 19 – 26 and 5 retrospective studies 27 – 31 of CyA used as single agent or in combination.…”

Section: Resultsmentioning

confidence: 99%

“… 32 – 34 In the setting of secondary ITP, a recent study including 83 adult patients with ITP associated with connective tissue disease showed that CyA was inferior to rituximab in terms of response rates (82% versus 54% at 6 months). 28 …”

Section: Resultsmentioning

confidence: 99%

“…ITP reports included patients with relapsed or refractory disease, and the overall response to CyA was about 65% (180/278 patients, case reports excluded), without differences among pediatric and adult settings. [20][21][22][23][24][25][27][28][29] Regarding combined regimens, the association of CyA and recombinant thrombopoietin (rTPO) versus rTPO single agent yields similar response rates (over 80%), but fewer relapses (29% versus 88% at 3 months). 21 In a clinical trial of 20 adult ITP patients, the association of CyA plus steroids and rituximab achieved a relapse-free survival of 92% and 76% at 12 and 24 months, respectively.…”

Section: Review Of the Literaturementioning

confidence: 99%

“…[32][33][34] In the setting of secondary ITP, a recent study including 83 adult patients with ITP associated with connective tissue disease showed that CyA was inferior to rituximab in terms of response rates (82% versus 54% at 6 months). 28 AIHA studies mainly consisted of case reports [12][13][14][15][16][17][18] with only one retrospective cohort study including relapsed or refractory subjects. 30 Overall response rate was about 70% (51/73, case reports excluded).…”

Section: Review Of the Literaturementioning

confidence: 99%

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Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers

Fattizzo

Cantoni

Giannotta

et al. 2022

Therapeutic Advances in Hematology

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Background: Immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) show good responses to frontline steroids. About two-third of cases relapse and require second-line treatment, including rituximab, mainly effective in AIHA, and thrombopoietin-receptor agonists (TPO-RAs) in ITP, while the use of splenectomy progressively decreased due to concerns for infectious/thrombotic complications. For those failing second line, immunosuppressants may be considered. Objectives: The aim of this study was to evaluate the efficacy of cyclosporine treatment in patients with ITP and AIHA. Design: In this retrospective study, we evaluated the efficacy and safety of cyclosporine A (CyA) in ITP ( N = 29) and AIHA ( N = 10) patients followed at two reference centers in Milan, Italy. Methods: Responses were classified as partial [Hb > 10 or at least 2 g/dl increase from baseline, platelets (PLT) > 30 × 109/l with at least doubling from baseline] and complete (Hb > 12 g/dl or PLT > 100 × 109/l) and evaluated at 3, 6, and 12 months. Treatment emergent adverse events were also registered. Results: The median time from diagnosis to CyA was 35 months (3–293), and patients had required a median of 4 (1–8) previous therapy lines. Median duration of CyA was 28 (2–140) months and responses were achieved in 86% of ITP and 50% of AIHA subjects. Responders could reduce or discontinue concomitant treatment and resolved PLT fluctuations on TPO-RA. CyA was generally well tolerated, and only two serious infectious complications in elderly patients on concomitant steroids suggesting caution in this patient population. Conclusion: CyA may be advisable in ITP, which is not well controlled under TPO-RA, and in AIHA failing rituximab, particularly if ineligible in clinical trial.

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Description of therapeutic strategies in severe systemic lupus erythematosus-associated immune thrombocytopenia: a retrospective cohort study of response and relapse

Cimé-Aké,

Barrera-Vargas,

Demichelis-Gómez

et al. 2024

Clin Rheumatol

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Rituximab or cyclosporin in refractory immune thrombocytopenia secondary to connective tissue diseases: a real-world observational retrospective study

Cited by 11 publications

References 20 publications

Risk factors of disease flares in a Chinese lupus cohort with low-grade disease activity

Risk factors of disease flares in a Chinese lupus cohort with low-grade disease activity

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers

Description of therapeutic strategies in severe systemic lupus erythematosus-associated immune thrombocytopenia: a retrospective cohort study of response and relapse

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