IgG4-Related Hypertrophic Pachymeningitis

Leptomeningeal form of Immunoglobulin G4-related hypertrophic meningitis with perivascular spread: a case report and review of the literature

“…Meningeal biopsy is the gold standard for establishing the diagnosis of IgG4-related disease [15]. Diagnostic criteria for IgG4-related pachymeningitis are based on three characteristic histological findings: lymphoplasmacytic infiltration of IgG4-positiveplasma cells, storiform fibrosis, and obliterative phlebitis.…”

“…There are no single immunohistochemical features that are pathognomonic for this disease. However, a cutoff value of 10 IgG4 + plasma cells/HPF, and an IgG4+/IgG+ plasma cell ratio > 40%, are recognized as sufficient for the diagnosis of IgG4-related disease of the meninges [15]. …”

A case report of atypical long segmental thoracic hypertrophic pachymeningitis with ossification of ligamentum flavum and literature review

“…According to etiology, HP is classified as idiopathic and secondary type. Autoimmune disorders was identified as secondary causes of HP in previous reports, such as Rheumatoid arthritis [1], Multifocal fibro sclerosis [2], Wegener's granulomatosis [3], Neuro-Behçet's disease [4], sarcoidosis [5], ANCA related diseases [6][7][8], IgG4 related diseases [9][10][11][12]23] and antiphospholipid syndrome [13]. Some infectious diseases caused by bacteria [14,37], fungi [15] and Spirochete [16,17] has been reported associated with HP as well.…”

Traitement de la maladie associée aux IgG4 en 2014 : mise au point et revue de la littérature

“…Le consensus japonais propose une dose de 0,6 mg/kg/j [5], alors que les équipes Nord Améri-caines proposent une dose fixe de 40 mg/jour [35]. L'intérêt des bolus de corticoïdes, réalisés dans certains cas d'atteintes rénales ou du système nerveux central, n'a pas été évalué [36,37].…”