CASE REPORT

CD8⁺ T lymphocytes infiltrate predominantly in the inflammatory foci of MPO-ANCA-positive thoracic hypertrophic pachymeningitis in a patient with HLA-A24

Authors

Tadashi Nakamura¹, Kei Hirakawa², Syu-ichi Higashi¹, Kunihiko Tomoda³, Michishi Tsukano³, Ken-ichi Iyama⁴ and Terumi Sakae²

1. Section of Internal Medicine and Rheumatology, Kumamoto Center for Arthritis and Rheumatology, 1-15-7 Kuhonji, Kumamoto 862-0976, Japan
2. Department of Orthopaedic Surgery, Kumamoto Orthopaedic Hospital, Kumamoto, Japan
3. Section of Orthopaedics and Rheumatology, Kumamoto Center for Arthritis and Rheumatology, Kumamoto, Japan
4. Department of Surgical Pathology, Kumamoto University Graduate School of Medical Sciences, Kumamoto, Japan

Received:

29 June 2006

Accepted:

25 October 2006

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Abstract

Hypertrophic pachymeningitis (HP) is extremely rare and an inflammatory process that thickens the dura mater. A 59-year-old Japanese woman developed backache, became paraplegic, and magnetic resonance imaging revealed diffuse thickening of the thoracic dura mater encompassing the spinal cord. Although a test for myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA) was shown to be positive, vasculitis was not found and CD8+ T lymphocytes that predominated in the inflammatory foci. Both interleukin (IL)-2 and IL-6 were markedly elevated in not only sera but also cerebrospinal fluids, very much higher in the latter. Human leukocyte antigen (HLA) typing revealed A24 positivity, suggesting this molecule was interacting with CD8+ T lymphocytes. It was suggested that immunological disharmony and autoimmunity would play a pivotal role in the development of HP under genetic background of HLA-A24, and HP would be one feature of multiple organ involvement in ANCA-associated diseases.

Key words

CD8⁺ T cells - Human leucocyte antigen (HLA) A24 - HLA-B51 - Hypertrophic pachymeningitis (HP) - Myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA)