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Evaluation of the safety, efficacy, and mechanism of action of obexelimab for the treatment of patients with IgG4-related disease: an open-label, single-arm, single centre, phase 2 pilot trial.
Lancet Rheumatol
August 2023
Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:
Background: Obexelimab is a bifunctional, non-cytolytic, humanised monoclonal antibody that binds CD19 and Fc gamma receptor IIb to inhibit B cells, plasmablasts, and CD19-expressing plasma cells. We aimed to evaluate the safety, clinical efficacy, and pharmacodynamic effects of obexelimab in patients with active IgG4-related disease.
Methods: We conducted an open-label, single-arm, single centre, phase 2 pilot trial at the Massachusetts General Hospital in Boston, MA, USA.
Obexelimab in IgG4-related disease: B-cell inhibition as a novel therapeutic approach.
Lancet Rheumatol
August 2023
Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Berlin 10117, Germany; Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany; Berlin Institute of Health, Berlin, Germany. Electronic address:
[IgG4-related disease: A proteiform pathology with frequent chest manifestations].
Rev Mal Respir
December 2023
Service de médecine Interne, hôpital La Timone, APHM, Aix-Marseille Université, 13005 Marseille, France. Electronic address:
Introduction: While IgG4-related disease (IgG4-RD) was initially described in the early 2000s, its polymorphic clinical manifestations were previously reported under different names ; they have in common the presence of IgG4+ oligoclonal plasma cells and fibrosis.
State Of The Art: Ruling out certain differential diagnoses, the diagnosis of IgG4-RD is based on a bundle of clinical, biological and histological features. Chest involvement is variable and can affect the mediastinum, bronchi, parenchyma, pleura and/or, more rarely, bones and (pericardium, aorta, coronary…) vascular structures.
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