Hypertrophic pachymeningitis as an unusual cause of headache and sphincter dysfunction in systemic lupus erythematosus
  • Soukaina Oumlil
    Department of Internal Medicine, University Hospital Mohammed VI, Marrakesh, Morocc
  • Mouna Zahlane
    Department of Internal Medicine, University Hospital Mohammed VI, Marrakesh, Morocc
  • Lamiaa Essaadouni
    Department of Internal Medicine, University Hospital Mohammed VI, Marrakesh, Morocc

Keywords

lupus, headache, urinary retention, Hypertrophic pachymeningitis

Abstract

Hypertrophic pachymeningitis (HP) is an uncommon condition characterised by focal or diffuse thickening of the dura mater. An increasing number of cases have been reported of its association with underlying connective tissue diseases. It is a rare complication in systemic lupus erythematosus (SLE) and might be the initial and sole clinical manifestation. We report a case of a 21-year-old man presenting with febrile meningeal syndrome and sphincter dysfunction. Physical examination showed malar rash and joint pain. Biological assessment revealed a regenerative normocytic normochromic anaemia, a leucopenia and a lymphopenia. The 24-hour urine protein was positive at 0.6 g. Immunological evaluation revealed positive antinuclear, anti-Sm and anti-dsDNA antibodies. Brain and spinal magnetic resonance imaging showed hypertrophic pachymeningitis. Cerebrospinal fluid biochemistry was within normal limits. Renal biopsy revealed a mesangial proliferative lupus nephritis. The diagnosis of SLE with neurologic and renal involvement was established, and the patient was treated with intravenous methylprednisolone pulse, followed by oral prednisone in association with azathioprine and hydroxychloroquine. Considering the persistence of symptoms and MRI lesions after 6 months, a treatment with rituximab was initiated with good evolution.

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References

  • Brüggemann N, Gottschalk S, Holl-Ulrich K, Stewen J, Heide W, Seidel G. Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology. J Neurol Neurosurg Psychiatry 2010;81:294–298.
  • Lee YC, Chueng YC, Hsu SW, Lui CC. Idiopathic hypertrophic cranial pachymeningitis: case report with 7 years of imaging follow-up. AJNR Am J Neuroradiol 2003;24:119–123.
  • Prabhakar S, Bhatia R, Lal V, Singh P. Hypertrophic pachymeningitis: varied manifestations of a single disease entity. Neurol India 2002;50:45–52.
  • Riku S, Hashizume Y, Yoshida M, Riku Y. Is hypertrophic pachymeningitis a dural lesion of IgG4-related systemic disease? Rinsho Shinkeigaku 2009;49:594–596.
  • Jang Y, Lee S-T, Jung K-H, Chu K, Lee SK. Rituximab treatment for idiopathic hypertrophic pachymeningitis. J Clin Neurol 2017;13:155–161.
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    Published: 2023-09-15
    Issue: 2023: Vol 10 No 10 (view)


    How to cite:
    1.
    Oumlil S, Zahlane M, Essaadouni L. Hypertrophic pachymeningitis as an unusual cause of headache and sphincter dysfunction in systemic lupus erythematosus. EJCRIM 2023;10 doi:10.12890/2023_004035.