Systemic Lupus Erythematosus and Thrombotic Thrombocytopenia Purpura: A Refractory Case Without Lupus Activity

Boyero, Raimundo García; Esteve, Eva Mas; Esteve, Maria Mas; Perseguer, M. Magdalena Millá; Buades, J.; Fabregat, J.; Ferrando, G; Serrano, Miguel Ángel Belmonte

doi:10.1016/j.reumae.2012.11.006

Cited by 6 publications

(1 citation statement)

References 12 publications

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“…However, although there are case reports and single-arm reports reporting the effect of RTX on CTDassociated TTP/TMA [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30], with some reports of exacerbations [31], there are no studies comparing an RTX-intervention group to a control group. In our department, RTX has been administered for CTDassociated refractory TTP/TMA after obtaining ethics committee approval and written consent.…”

Section: Introductionmentioning

confidence: 99%

Efficacy and safety of rituximab in connective tissue disease-associated thrombotic thrombocytopenic purpura/thrombotic microangiopathy

Ohkubo

Nakayamada

Hanami

et al. 2023

Preprint

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Background This study examined the efficacy and safety of Rituximab (RTX) treatment in connective tissue disease (CTD)-associated thrombocytopenic purpura (TTP) and thrombotic microangiopathy (TMA), using historical controls as comparators. Methods Patients who were admitted to our department from March 1, 2013, to March 31, 2021, and diagnosed with CTD-associated TTP/TMA refractory to plasma exchange were included in the retrospective study. A patient with treatment-resistant disease was treated with RTX in addition to high-dose glucocorticoid (GC) therapy (GC + RTX). As historical controls, we selected patients with CTD-associated TTP/TMA who were admitted to our center and treated with GC and immunosuppressants (IS) such as cyclophosphamide. The primary endpoint was the survival rate at 52 weeks after initiation of treatment. Results Fifteen patients were enrolled in the study (GC + RTX). As a control group, 11 patients were enrolled in the same manner (GC + IS). There were no significant differences in age or sex or laboratory tests between the two groups. The primary endpoint of survival rate was higher in the GC + RTX group than in the GC + IS group. In the immunophenotyping analysis before treatment, among all subsets of immune cells, only plasmocytes were elevated in TTP patients compared to healthy controls. The proportion ofplasmocytes correlated with serum markers, suggesting increased B cell differentiation, which was markedly decreased after RTX treatment. Conclusion In CTD-associated TTP/TMA, the addition of RTX to plasma exchange and GC therapy may improve B cell-based pathology and survival.

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Section: Introductionmentioning

confidence: 99%

Efficacy and safety of rituximab in connective tissue disease-associated thrombotic thrombocytopenic purpura/thrombotic microangiopathy

Ohkubo

Nakayamada

Hanami

et al. 2023

Preprint

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Heparin-Related Thrombocytopenia Triggered by Severe Status of Systemic Lupus Erythematosus and Bacterial Infection

Suzuki

Nakajima

Ando

et al. 2016

Case Reports in Rheumatology

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A patient with severe lupus nephritis developed thrombocytopenia during treatment with high-dose steroids. In addition to viral- or disease-induced cytopenia, the pathology was believed to arise from diverse contributing factors, such as thrombotic microangiopathy and heparin-related thrombocytopenia (HIT). By combining plasma exchange therapy and intravenous cyclophosphamide, we successfully controlled the SLE activity and improved the thrombocytopenia. An antecedent bacterial infection or SLE activity is believed to have contributed to the concurrent HIT.

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Prospects for Anti-B-Cell Therapy in Immuno-Inflammatory Rheumatic Diseases

Насонов

Бекетова²,

Ананьева³

et al. 2019

Naučno-praktičeskaâ revmatologiâ

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Systemic Lupus Erythematosus and Thrombotic Thrombocytopenia Purpura: A Refractory Case Without Lupus Activity

Cited by 6 publications

References 12 publications

Efficacy and safety of rituximab in connective tissue disease-associated thrombotic thrombocytopenic purpura/thrombotic microangiopathy

Efficacy and safety of rituximab in connective tissue disease-associated thrombotic thrombocytopenic purpura/thrombotic microangiopathy

Heparin-Related Thrombocytopenia Triggered by Severe Status of Systemic Lupus Erythematosus and Bacterial Infection

Prospects for Anti-B-Cell Therapy in Immuno-Inflammatory Rheumatic Diseases

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