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A case of rheumatoid meningitis positive for perinuclear antineutrophil cytoplasmic antibody
  1. M FUNAUCHI,
  2. B S YOO,
  3. M SUGIYAMA,
  4. S IKOMA,
  5. M OHNO,
  6. K KINOSHITA,
  7. K HAMADA,
  8. A KANAMARU
  1. Third Department of Internal Medicine
  2. Kinki University School of Medicine
  3. 377–2 Ohno-Higashi
  4. Osaka-Sayama 589–8511
  5. Osaka, Japan
  1. funauchi{at}med.kindai.ac.jp

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Rheumatoid meningitis (RM) is a pachymeningitis rarely occurring in rheumatoid arthritis (RA). The pathophysiology of RM is not clear, though a diffuse vasculitis in the dura mater has been shown in some patients.1 Here we present a patient with RM who had a raised serum level of perinuclear antineutrophil cytoplasmic antibody (P-ANCA), which is known to be associated with vasculitic diseases.

A 72 year old woman was admitted to our hospital because of headache. Twenty two years before she had been diagnosed as having RA and treated with non-steroidal anti-inflammatory drugs, without complete remission. Seven months before admission, polyarthralgia deteriorated and a numbness of the hands and feet and a low grade fever occurred, and she lost 10 kg in 2 months. She was given prednisolone 15 mg/day and her symptoms disappeared in a few weeks; the dose of prednisolone was tapered to 5 mg/day over three months. Five weeks before admission a left temporal headache occurred and gradually worsened; then she was admitted to the hospital. On physical examination, there were paraesthesia in the hands and feet, but no meningeal signs, papilloedema, visual field defects, cranial nerve palsies, ataxia or pathological reflexes were present. In laboratory studies, urine analysis and blood chemistry were normal. Table 1 shows the serological findings. Analysis of cerebrospinal fluid was normal and multiple cultures were negative for micro-organisms. Magnetic resonance imaging (MRI) of the brain disclosed a diffusely thickened dura mater along the left cerebral hemisphere and the cerebellar tentorium was enhanced after gadolinium pentetic acid (Gd-DTPA) administration (figs 1A and B). Biopsy of the dura mater was refused by the patient. After various causes of pachymeningitis2-4such as sarcoidosis, Wegener's granulomatosis, malignant lymphoma, and a number of infectious diseases were excluded, a diagnosis of RM was made. The dose of prednisolone was increased to 50 mg/day, and the headache disappeared in two weeks. Raised levels of rheumatoid factor (2390 fell to 153 IU/ml ) and P-ANCA (65 fell to <10 EU) gradually normalised in eight weeks. Brain MRI performed three months later showed no dural thickening (figs 1C and D).

Table 1

Blood count and serological findings

Figure 1

Magnetic resonance imaging after administration of gadolinium on admission (A, B) and six months after treatment (C, D). T1 weighted images are shown. Arrows represent the thickened dura mater along the left cerebral hemisphere and the cerebellar tentorium. (A, C) Horizontal section; (B, D) coronal section.

Recent development of MRI has helped the diagnosis of RM greatly.5 Because enhancement of MRI by administration of Gd-DTPA implies disruption of the brain-blood barrier,6 it was suspected that this case was complicated by RM.

Although it has been reported that some neurological complications in RA are related to vasculitis,6 7 there have been some investigators who could not show the existence of vasculitis in patients with RM.8 Demonstration of vasculitis in RM may depend on the phase and extent of the pathological changes of RM.

P-ANCA is often detected in systemic vasculitic diseases, such as polyarteritis nodosa and pauci-immune necrotising crescentic glomerulonephritis,9 but it has been reported that the sensitivity and specificity of P-ANCA for vasculitis are not high. Because vasculitis occurs in rheumatic diseases, which are often mediated by immune complexes, P-ANCA in this case may result from non-specific polyclonal B cell activation. On the other hand, it has also been reported that the frequency of vasculitic involvement was significantly high in a P-ANCA positive subgroup of patients with RA.10 However, the number of the cases in which pachymeningitis is shown by MRI or pathological analysis are few, and the serum P-ANCA was determined and followed up in the clinical course. Therefore, accumulation of cases of RM in which serum P-ANCA is determined may be helpful in understanding the pathogenesis of RM and the histochemical analysis of this antibody in the dura mater.

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