P-ANCA cranial pachymeningitis: a case report.
Pachymeningitis is an inflammatory process that thickens the dura mater. This disease has various etiologies including infectious, neoplastic, or autoimmune diseases. We present the case of a patient who developed cranial pachymeningitis with a clinical and biological picture suggestive of a neurological form of vasculitis. A 51-year-old woman developed rhinitis, otitis media, headaches, and deterioration of her condition after a course of recombinant hepatitis B vaccine. After a booster dose of the vaccine, she developed unilateral visual loss and impairment of multiple cranial nerves. Blood analysis showed inflammation and presence of antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA). Cranial magnetic resonance imaging (MRI) showed pachymeningitis. A complete remission was obtained with immunosuppressive therapy. The initial clinical presentation and subsequent remission under immunosuppressive therapy were suggestive of a vasculitis with nervous system involvement. Though vasculitis was not proven histologically in this patient, we believe that MPO-ANCA-related autoimmunity provoked the patient's disease as already reported in similar cases. As pachymeningitis is a fibrosing process, early recognition and treatment of an autoimmune etiology, even in the absence of previous pulmonary or renal involvement, is required to prevent definitive neurological impairment.
Jacobi
D
D
Service de Medecine Interne A, CHU Bretonneau, Centre Hospitalier Universitaire, 2 bis, boulevard Tonnelle, Tours, 37000, France.
Maillot
F
F
Hommet
C
C
Arsène
S
S
Cottier
J P
JP
Lamisse
F
F
Guillevin
L
L
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood / Q000276:immunology*; D004388:Dura Mater / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000209:etiology* / Q000276:immunology* / Q000473:pathology; D008875:Middle Aged; D009195:Peroxidase / Q000276:immunology; D020293:Vasculitis, Central Nervous System / Q000150:complications* / Q000276:immunology* / Q000473:pathology
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase