Association of IgG4-related disease with recurrent hemorrhagic and ischemic strokes

초록

Background and aims: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated inflammatory disease that characterized by an inflammatory reaction rich in IgG4-positive plasma cells, affecting multiple organs. Depending on the site of the tissue infiltration, IgG4-RD can present as a consequence of obstruction or compression with the pancreas, biliary tree and urinary symptoms and hydronephrosis. IgG4-RD has also been linked to arterial inflammation. However, there are few case reports presenting recurrent stroke including both hemorrhagic and ischemic strokes. Methods: This report describes a case who was diagnosed with IgG4-RD, having intracranial hemorrhage and a subsequent acute ischemic stroke of undetermined cause. Results: A 79-year-old woman presented with headache and both lower limbs weakness and two weeks later she was admitted with acute attacks of mild left upper limb sensory-motor monoparesis. Indirect immunohistochemistry assay showed elevated level of IgG4, labeling neurons of the central nervous system, suggesting an immunological process possibly affecting vascular structures. Conclusions: Although a definitive link between IgG4-RD and stroke has not been established, IgG4-RD should be considered as an etiology in patients with both hemorrhagic and ischemic strokes.