Clinical presentation, treatment and outcome of IgG4-related pachymeningitis: From a national case registry and literature review

• Published in: Seminars in arthritis and rheumatism 2019-12, Vol.49 (3), p.430-437
• Main Authors: Melenotte, Cléa, Seguier, Julie, Ebbo, Mikael, Kaphan, Elsa, Bernit, Emmanuelle, Saillier, Laurent, Audoin, Bertrand, Feyeux, Delphine, Daniel, Laurent, Roche, Pierre-Hugues, Graillon, Thomas, Dufour, Henry, Boutière, Clémence, Girard, Nadine, Closs-Prophette, Fabienne, Guillaud, Constance, Tieulié, Nathalie, Regent, Alexis, Harlé, Jean Robert, Hamidou, Mohamed, Mekinian, Arsène, Grados, Aurélie, Schleinitz, Nicolas
• Format: Article
• Language: English
• Subjects: Hypertrophic meningitis; IgG4-related disease; Pachymenigitis
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2019.05.003

Pachymeningitis is rare, either idiopathic or secondary to inflammatory disorders, after tumoral, surgical or infectious causes have been excluded. The fibroinflammatory IgG4-related disease is one of the etiologies of pachymeningitis with only few cases reported yet. From a single referral regional center, we evaluated the frequency of IgG4-related disease as the cause of inflammatory pachymeningitis in 10% of cases. From a National case registry of IgG4-related disease the pachymeningitis frequency was 4.1%. We report eight new cases with cranial, spinal or both involvements and a literature review of 46 pathological proven cases. We observed that IgG4-related pachymeningitis is in most cases not associated to extra-neurological manifestations of the disease. Only 27% of spinal and 40% of cranial IgG4-related pachymeningitis are associated with other disease localizations. First line treatment strategies included surgery and steroids. The use of immunosuppressants or rituximab was necessary in 18% of spinal and 54% of cranial localizations. Some patients remained with sequellae and clinical and/or radiological improvement can be difficult to obtain.