title: Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti-neutrophil cytoplasmic antibody-associated vasculitis.
abstract: OBJECTIVE
  To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
  
  
  METHODS
  We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP.
  
  
  RESULTS
  Of 39 patients with AAV, seven (17.9%) had associated HP. All patients with HP were classified as having granulomatosis with polyangiitis (GPA), whereas only five of 32 patients without HP were diagnosed as having GPA (P < 0.0001). The frequencies of myeloperoxidase (MPO)-ANCA and proteinase 3-ANCA positivity in patients with HP were equivalent, while MPO-ANCA positivity was obviously dominant in patients without HP. HP occurred as the initial clinical episode of AAV in three patients (7.7% of all AAV). Frequent significant characteristics of patients with HP were headache, cranial neuropathy and paranasal involvement (P < 0.05), and histopathological findings from paranasal involvement were useful for the diagnosis of GPA in some patients with HP. Combination therapy of corticosteroid and an immunosuppressant, such as methotrexate, cyclophosphamide or rituximab, was effective for achieving remission and improving radiographic findings of HP.
  
  
  CONCLUSIONS
  AAV is a common cause of HP; epidemiological features of AAV patients with HP are different from those of patients without HP. Additionally, HP impacts diagnosis because it may be an initial clinical sign of AAV.
authors.affiliation: Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Shimojima
authors.affiliation: Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Dai
authors.initials: D
authors.lastname: Kishida
authors.affiliation: Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.|Intractable Disease Care Center, Shinshu University Hospital, Matsumoto, Japan.
authors.forename: Akiyo
authors.initials: A
authors.lastname: Hineno
authors.affiliation: Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.|Institute for Biomedical Sciences, Shinshu University, Matsumoto, Japan.
authors.forename: Masahide
authors.initials: M
authors.lastname: Yazaki
authors.affiliation: Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.|Institute for Biomedical Sciences, Shinshu University, Matsumoto, Japan.
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Sekijima
authors.affiliation: Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.|Institute for Biomedical Sciences, Shinshu University, Matsumoto, Japan.
authors.forename: Shu-Ichi
authors.initials: SI
authors.lastname: Ikeda
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D004359:Drug Therapy, Combination; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007564:Japan / Q000453:epidemiology; D008297:Male; D008581:Meningitis / Q000175:diagnosis / Q000453:epidemiology*; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D007166:Immunosuppressive Agents
keywords: ANCA-associated vasculitis; anti-neutrophil cytoplasmic antibody; granulomatosis with polyangiitis; granulomatous lesion; hypertrophic pachymeningitis