| ObjectiveTo investigate clinical manifestations,imaging features,laboratory tests,treatment and prognosis of myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis.MethodClinial data of 4 cases diagnosed with myeloperoxidase antineutrophil cytoplasmic antibody related hypertrophic pachymeningitis in our hospital and other 23 cases reported in detail in the literature were analysed retrospectively.Results1.The incidence of myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis is higher in East Asia,Japan and China in particular.The dura mater and upper respiratory tract were mostly involved by lesions,manifested as headache to a different degree,multiple cranial neuropathies,and clinical symptoms of upper respiratory tract involved(eg,sinusitis,otitis media or mastoiditis,saddle nose deformity,retrobulbar mass,etc.).The kidney was rarely involved.2.Levels of inflammatory biomarkers,titers of MPO-ANCA and concentrations of cerebrospinal fluid proteins elevated to a different degree in laboratory tests.Contrast-enhanced magnetic resonance imaging(MRI)scan was highly specific(100%),manifested as dura mater thickened and obviously enhanced.Pathology showed severe thickening of the dura mater with chronic inflammation,7 cases were performed dura mater biopsy,of which 4 case(57.1%)was compatible with granulomatosis with polyangiitis.3.The main therapeutic drugs were glucocorticoid and immunosuppressants,almost all patients had good prognosis after active treatment.ConclusionMPO-ANCA associated hypertrophic pachymeningitis may be a central nevous system-limited granulomatosis with polyangiitis.Rheumatologists and neurologists must reinforce understandings of this disease,so that taken it into consideration when encountered elderly patients with headache and multiple cranial neuropathies.Enhanced MRI is the priority for diagnosis,and dural biopsy when necessary. |
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