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Pachymeningitis in microscopic polyangiitis (MPA): a case report and a review of central nervous system involvement
H Kono1, S Inokuma, H Nakayama
1Department of Allergy and Immunological Diseases, Tokyo Metropolitan Komagome Hospital, Japan.
Abstract:
A case of microscopic polyangiitis (MPA) with pachymeningitis is described. The patient had renal, skin, gallbladder and peripheral nervous system involvement, simultaneously with pachymeningitis. Necrotizing glomerulonephritis with crescent formation, and necrotizing small vessel vasculitis in the kidney and skin were confirmed by biopsy. A highly elevated titer of antineutrophil cytoplasmic antibody for myeloperoxidase (MPO-ANCA) was observed. All of the clinical and laboratory abnormalities improved with high-dose pulse and conventional steroid therapy. The literature on central nervous system involvement in MPA and perinuclear-ANCA (p-ANCA)-related vasculitis is reviewed. This case serves to emphasize that pachymeningitis can occur as one of the features of MPA.
Insights
Microscopic polyangiitis (MPA) can present with pachymeningitis, affecting multiple organs including the kidneys and nervous system. This case highlights MPA
Area of Science:
- Rheumatology
- Neurology
- Nephrology
Background:
- Microscopic polyangiitis (MPA) is a rare autoimmune disease characterized by inflammation of small blood vessels.
- Central nervous system (CNS) involvement in MPA is uncommon, with pachymeningitis (inflammation of the dura mater) being a particularly rare manifestation.
- Antineutrophil cytoplasmic antibodies (ANCAs), especially those against myeloperoxidase (MPO-ANCA), are key diagnostic markers for MPA.