Trial reportBMC musculoskeletal disorders2010
Effects of rituximab in two patients with dysferlin-deficient muscular dystrophy.
Trial report in BMC musculoskeletal disorders, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 38 citations in OpenAlex.
- Progression to Loss of Ambulation Among Patients with Autosomal Recessive Limb-girdle Muscular Dystrophy: A Systematic Review.Journal of neuromuscular diseases · 2022Pooled it
- 1α,25(OH)(2)-Vitamin D3 increases dysferlin expression in vitro and in a human clinical trial.Molecular therapy : the journal of the American Society of Gene Therapy · 2012Trial
- Dysferlinopathies: phenotypic study of a Moroccan series of 28 cases.Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2026Article
- The Clinicopathological Distinction between Immune-Mediated Necrotizing Myopathy and Limb-Girdle Muscular Dystrophy R2: Key Points to Prevent Misdiagnosis.Journal of clinical medicine · 2022Article
- Rituximab as a treatment for human immunodeficiency virus-associated nemaline myopathy: What does the literature have to tell us?World journal of clinical cases · 2022Article
- Role of Immunoglobulins in Muscular Dystrophies and Inflammatory Myopathies.Frontiers in immunology · 2021Review
- Membrane Stabilization by Modified Steroid Offers a Potential Therapy for Muscular Dystrophy Due to Dysferlin Deficit.Molecular therapy : the journal of the American Society of Gene Therapy · 2018Article
- Annexin A2 links poor myofiber repair with inflammation and adipogenic replacement of the injured muscle.Human molecular genetics · 2017Article
- Adaptive Immune Response Impairs the Efficacy of Autologous Transplantation of Engineered Stem Cells in Dystrophic Dogs.Molecular therapy : the journal of the American Society of Gene Therapy · 2016Article
- AAV.Dysferlin Overlap Vectors Restore Function in Dysferlinopathy Animal Models.Annals of clinical and translational neurology · 2015Article
- Limb-girdle muscular dystrophies: where next after six decades from the first proposal (Review).Molecular medicine reports · 2014Review
- From innate to adaptive immune response in muscular dystrophies and skeletal muscle regeneration: the role of lymphocytes.BioMed research international · 2014Review
- Influence of immune responses in gene/stem cell therapies for muscular dystrophies.BioMed research international · 2014Review
- The effects of MyD88 deficiency on disease phenotype in dysferlin-deficient A/J mice: role of endogenous TLR ligands.The Journal of pathology · 2013Article
- Dysferlinopathy course and sportive activity: clues for possible treatment.Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology · 2011Article
- A dual acting compound releasing nitric oxide (NO) and ibuprofen, NCX 320, shows significant therapeutic effects in a mouse model of muscular dystrophy.Pharmacological research · 2011Article
- Muscle membrane repair and inflammatory attack in dysferlinopathy.Skeletal muscle · 2011Article
- Effects of Prednisone on a Patient with Dysferlinopathy Assessed by Maximal Voluntary Isometric Contraction: Alternate-Day Low-Dose Administration for a 17-Year Period.Case reports in neurologyArticle
- Translational research and therapeutic perspectives in dysferlinopathies.Molecular medicine (Cambridge, Mass.)Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe administration of rituximab (RTX) in vivo results in B-cell depletion, but evidence for multiple mechanisms of action have been reported. Surprisingly, B cell depletion produced a response in patients with polymyositis, which is characterized as a T cell-mediated autoimmune disorder with biopsy findings similar to Miyoshi myopathy (MM). Indeed, in dysferlinopathies, there is evidence of immune system involvement including the presence of muscle inflammation and a down regulation of the complement inhibitory factor, CD55.
methodsTwo patients were treated with four weekly infusions of RTX 375 mg/m2. To measure the improvement in muscle strength after treatment, the isometric hand grip maximal voluntary contraction (MVC) was measured by load cell four times during treatment, and again after one year. In order to assess the reproducibility of our grip assessment, we determined the hand MVC analysis in 16 healthy subjects. Moreover, we measured the number of B cells present in patients by flow cytometric analysis during the course of treatment.
resultsThe analysis of B cell number during the course of treatment showed that CD20- and CD19-positive cells were depleted to 0-0.01%. The decrease in B cells was followed by an improvement in the mobility of the pelvic and shoulder girdles as shown by the MRC%. The MVC values of both patients began at values lower than normal whereas during treatment patients had improved percentage of muscle strength. The strength peak in both patients coincided with the minimum B cell values. There were no severe adverse events associated with an infusion of RTX.
conclusionWe consider the increase in muscle strength observed in both treated patients to be a consequence of their treatment with RTX. To our knowledge, these are the first cases of increased muscle strength in patients with MM. Furthermore, the results of this study indicate that B cell depletion with RTX may be useful in the treatment of patients affected by MM, suggesting a possible role for B cells in the pathophysiology of this muscle disorder.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.