Abstract

ROVERANO, Susana et al. IgG4-related hypertrophic pachymeningitis: communication of eleven other cases and literature review. Rev Cuba Reumatol [online]. 2019, vol.21, suppl.1  Epub Dec 01, 2019. ISSN 1817-5996.

Pachymeningitis is a rare disorder characterized by focal or diffuse thickening of the dura mater, being a potential manifestation of the disease related to IgG4. We present 11 patients, six men and five women, aged 39-73 years, who consulted for headache, visual disturbances, tinnitus, hearing loss, weight loss, salivary gland enlargement, dorsalgia, quadriplegia and cranial nerve involvement. Some of them presented elevation of the C-reactive protein or the erythrocyte sedimentation value, while the majority had normal serum levels of IgG4. All patients showed thickening of the dura in magnetic resonance imaging. The biopsy of dura mater, gallbladder, pituitary gland or lacrimal gland showed a lymphoplasmacytic infiltrate with or without storiform fibrosis, with more than 10 IgG4 (+) plasmocytes and an IgG4: IgG range that ranged from 20 to 60%. Patients received prednisone alone or with rituximab, methotrexate, cyclophosphamide or azathioprine, with favorable response.

Keywords : pachimeningitis; IgG4-related disease; vasculitis.

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