Hypertrophic pachymeningitis: a rare manifestation of IgG4 related disease

Hypertrophic pachymeningitis (HP) is a rare form of diffuse inflammatory disease that causes thickening the dura mater. It can involve cranial or spinal both. An increasingly well-known symptom IgG4-related illness, fibroinflammatory syndrome may affect almost any organ, hypertrophic (IgG4-RHP). estimated IgG4-RHP account for high proportion cases once considered idiopathic. Contrast magnetic resonance imaging (MRI) shows pachymeningeal enhancement. Serum IgG4 levels be elevated but are normal in most patients. However, patients have cerebrospinal fluid (CSF) index. Hence, CSF index could serve as less invasive diagnostic marker IgG4-RHP. Confirmation diagnosis by meningeal biopsy swirling “storiform” fibrosis with lymphocytic infiltrates, obliterate phlebitis and positive plasma cells. This case highlights dilemma gold standard which has many its own limitations. an alternate option when procedure contraindicated uninformative.