Abstract
Background Immunoglobulin G4 related disease (IgG4 RD) is a multi-system, immunologically-mediated disease discovered within the last two decades. Within the nervous system, a broad range of both central and peripheral nervous system involvement have been described in the literature. We aimed to systematically review the neurological manifestations of IgG4 RD.
Aim To identify the clinical manifestations, presenting symptoms and diagnostic methods for the neurological manifestations of IgG4 RD.
Methods We systematically reviewed the literature to identify all case reports of neurological manifestations of IgG4 RD. Data regarding neurological manifestations, non-neurological manifestations, clinical presentation and diagnostic methods were extracted.
Results We identified 393 patients from 297 articles meeting the inclusion criteria. The common neurological manifestations are hypertrophic pachymeningitis (39.4%), inflammatory orbital disease (37.2%), hypophysitis (17.3%), myelopathy (10.7%) and peripheral neuropathy (4.3%). The most common initial symptom is headache at 35.6%, followed by visual loss at 22.1%. Common concomitant non-neurological manifestations include pulmonary disease (airway and parenchymal lesions) (8.9%), lymphadenopathy (8.1%), salivary gland enlargement (7.9%), and pancreatitis (5.3%). Definite IgG4 RD criteria was met in 196 patients (49.9%) while probable criteria was met in 150 (38.2%) and possible in 47 (12.0%).
Conclusion Neurological manifestations of IgG4 RD most commonly occur as hypertrophic pachymeningitis, inflammatory orbital disease and hypophysitis. Headache and visual loss are the most common presenting complaints. Pulmonary disease, lymphadenopathy and salivary gland enlargement are the most commonly described concomitant non-neurological manifestations. Patients with these various manifestations should prompt consideration of IgG4 RD which requires histopathological confirmation for definitive diagnosis.
