@article {1593835,
	title = {The neurology of IGG4-related disease},
	journal = {J Neurol Sci},
	volume = {424},
	year = {2021},
	month = {2021 05 15},
	pages = {117420},
	abstract = {PURPOSE OF REVIEW: IgG4-related disease (IgG4-RD) is emerging as a fibro-inflammatory entity affecting multiple organs, including manifold neurologic manifestations. This review discusses general characteristics of IgG4-RD neurologic disease including epidemiology, histology, clinical picture and treatment approaches. RECENT FINDINGS: IgG4-RD is increasingly recognized as an important underlying pathophysiology in multiple disorders of neurologic interest, including orbital inflammation, infundibulo-hypophysitis, hypertrophic pachymeningitis, and even in rare cases CNS parenchymal disease and cranial vascular involvement. These were previously considered idiopathic and unrelated to any systemic disease but now known to share a common histopathology. New knowledge regarding the pathogenesis, clinical features and epidemiology of IgG4 is emerging, and new neurological manifestations continue to be described. Diagnostic progress includes CT-PET imaging, the use of flow cytometry for plasmablast quantification, and the use of reverse passive latex agglutination aiming to overcome the prozone phenomenon. Histopathologic confirmation of IgG4-RD remains the gold standard method of diagnosis but new diagnostic criteria for systemic and organ-specific disease are being proposed. Though glucorticoids remain the mainstay of therapy, relapses and incomplete recovery are frequent. Rituximab is a promising treatment in IgG4-RD that is severe, refractory or glucocorticoid dependent. Initiation of immunosuppression at an early stage of disease should be considered in order to avoid development of refractory fibrosis. SUMMARY: The current review emphasizes the neurologic manifestations of IgG4-RD.},
	keywords = {Autoimmune Diseases, Humans, Immunoglobulin G, Immunoglobulin G4-Related Disease, Meningitis, Neurology, Plasma Cells},
	issn = {1878-5883},
	doi = {10.1016/j.jns.2021.117420},
	author = {Saitakis, G and Chwalisz, B K}
}
