IgG4- and MPO-ANCA-associated hypertrophic pachymeningitis
*Correspondencia: Dr. Josep M. Aragonès Pascual. Servicio de Neurología. Consorci Hospitalari de Vic. Francesc Pla ‘el Vigatà’, s/n. E-08500 Vic (Barcelona).
E-mail: jmaragones@chv.cat
Introduction: Idiopathic hypertrophic pachymeningitis is a fibroinflammatory immune-mediated disease of the dura mater. Its diagnosis requires the preclusion of infectious, tumoral and other inflammatory diseases. In recent years new entities have been reported that can present with hypertrophic pachymeningitis, such as IgG4-associated disease and MPO-ANCA+ pachymeningitis, as a form of vasculitis limited to the central nervous system.
Case report: We describe the case of a 64 years-old male with headaches and cervicalgia, predominantly at night, and clinical signs and symptoms of spinal cord compression. Following the diagnosis of craniocervical hypertrophic pachymeningitis provided by the magnetic resonance imaging study, an aetiological study was conducted. Infectious and tumoral diseases were precluded. The clinical features did not show any systemic involvement and high levels of IgG4 and MPO-ANCA+ were found in the results of the analyses. The clinical signs and symptoms quickly improved following treatment with corticoids.
Conclusions: IgG4-related disease and MPO-ANCA-associated vasculitis limited to the central nervous system can account for a high percentage of the cases of hypertrophic pachymeningitis that were considered idiopathic, and their diagnosis requires a biopsy and a histological study.
Caso clínico Varón de 64 años con cefalea y cervicalgia de predominio nocturno y clínica de compresión medular. Tras el diagnóstico de paquimeningitis hipertrófica craneocervical facilitado por el estudio de resonancia magnética, se realizó un estudio etiológico. Se descartaron enfermedades infecciosas y tumorales. La clínica no mostraba afectación sistémica y en la analítica presentaba IgG4 elevada y MPO-ANCA+. Tras tratamiento con corticoides presentó una rápida mejoría de la clínica.
Conclusiones La enfermedad relacionada con IgG4 y la vasculitis asociada a MPO-ANCA limitada al sistema nervioso central pueden representar un alto porcentaje de las paquimeningitis hipertróficas que se consideraban idiopáticas, y su diagnóstico requiere biopsia y estudio histológico.