Background/Purpose:  Hypertrophic pachymeningitis (HP) is a rare, chronic and inflammatory disorder. HP is characterized by thickening of the cranial dura mater. The cause of HP was reported to be associated with some disorders such as infectious disease, connective tissue disease (CTD) and malignant tumor¹⁾. Recently, some reports showed that some patients with hypertrophic pachymeningitis may have myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive status²⁾. However, the clinical characteristics and specific treatments for CTD associated hypertrophic pachymeningitis remain elusive. Because of its low incidence of HP, clinical course such as symptom, laboratory data and treatment strategy was unclear. ^{1) 2)} Previous reports have shown that HP patients usually respond to glucocorticoid (GC), but not some patients were recurred during steroid tapering ³⁾. We extract the clinical features and treatment regimen, and investigate the clinical characteristics of HP. We examined HP patients in our institution in order to reveal the incidence of CTD complication and the efficacy of treatment.

Methods:  We enrolled the 6 patients (M/F: 2/4) with HP diagnosed and treated in our facility between 2000 and 2015. We extract the subjects as follow; initial symptom, underlying disorder, initial intervention and the efficacy of treatment.

Results: The mean age at diagnosis of HP was 43.7 years old (range; 18-73). The initial symptom of HP was headache in 5 cases, eye manifestation in 3 cases, fever in 2 cases, nausea in 1 case and tinnitus in 1 case. Underlying disorders complicated HP existed in 4 of 6 cases and all 4 cases diagnosed CTD (two only Sjögren’s syndrome, one mixed connective tissue disease and Sjögren’s syndrome, one granulomatosis with polyangitis). Performed meningeal biopsy was only 1 case. Infiltration of lymphocytes and anti-IgG4-positive plasma cells (IgG4/IgG≧40%), but we couldn’t diagnose IgG4-related disease. The intervention was conducted with glucocorticoid (GC) treatment in all HP patients. 3 cases were taken methylprednisolone-pulse. Two third of 6 HP patients had well responded to initial GC treatment. However two patients were refractory treatment. One case treated with methotrexate combined with GC, and another case cyclosporine A.

Conclusion:  In HP patients in our institution complicated with CTD, headache and eye manifestation often observed as initial symptoms. Several reports showed that serum IgG4 level was normal in IgG4-related HP as this case. Sjögren’s syndrome is most frequent complication in our hospital and these HP patients are refractoriness to GC. It is necessary for refractory HP patients to treat using immunosuppressive therapy with combined GC.

 References: 

1) Yonekawa T, et al. A nationwide survey of hypertrophic pachymeningitis in Japan. J Neurol Neurosurg Psychiatry. 2014 ;85(7):732-9

2) Yokoseki A, et al. Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody. Brain. 2014 ;137:520–36.

3) Iwanami M, et al. Orbital apex syndrome due to relapse during steroid tapering in a patient with MPO-ANCA-positive IgG4-related hypertrophic pachymeningitis. Rinsho Shinkeigaku. 2014;54(1):52-5.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-analysis-of-hypertrophic-pachymeningitis/