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Yamamoto et al1 recently reported the case of a patient with long-standing and relapsing IgG4-related disease (IgG4-RD), who developed secondary resistance to rituximab treatment but subsequently improved under abatacept infusions. This interesting observation drives important questions, regarding both IgG4-RD pathophysiology and abatacept mechanisms of action.
IgG4-RD is a systemic disease of unknown cause, affecting primarily middle-aged men, characterised by swelling of one or several organs (mainly pancreas, salivary and lachrymal glands, lymphadenopathy, retroperitoneal organs) with infiltration of IgG4-positive plasma cells and fibrosis of involved organs, and high serum IgG4 levels in half of the patients.2 ,3 Several diagnostic or classification systems have been proposed.4 ,5
Its pathophysiology is still ill-defined.6 IgG4-secreting plasmablasts are central players in the disease: they are the main infiltrating cells in affected organs, they are expanded in the circulation, are …
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