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Small-vessel vasculitis

 

Efficacy of rituximab for anti-neutrophil cytoplasmic antibody-associated hypertrophic pachymeningitis: a case series


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12

 

  1. Third Department of Internal Medicine, University of Yamanashi, and Centre for Clinical Immunology and Rheumatology, University of Yamanashi Hospital, Japan.
  2. Third Department of Internal Medicine, University of Yamanashi, and Centre for Clinical Immunology and Rheumatology, University of Yamanashi Hospital, Japan. dnakagomi@hotmail.co.jp
  3. Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan.
  4. Third Department of Internal Medicine, University of Yamanashi, and Centre for Clinical Immunology and Rheumatology, University of Yamanashi Hospital, Japan.
  5. Third Department of Internal Medicine, University of Yamanashi, and Centre for Clinical Immunology and Rheumatology, University of Yamanashi Hospital, Japan.
  6. Department of Rheumatology, National Hospital Organization Shimoshizu National Hospital, Chiba, Japan
  7. Centre for Allergy and Clinical Immunology, Japanese Red Cross Narita Hospital, Chiba, Japan.
  8. Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan.
  9. Department of Allergy and Clinical Immunology, Chiba University Hospital, and Centre for Allergy and Clinical Immunology, Japanese Red Cross Narita Hospital, Chiba, Japan.
  10. Centre for Allergy and Clinical Immunology, Japanese Red Cross Narita Hospital, Chiba, Japan.
  11. Department of Rheumatology, National Hospital Organization Shimoshizu National Hospital, Chiba, Japan.
  12. Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan.

CER12958
2020 Vol.38, N°2 ,Suppl.124
PI 0176, PF 0181
Small-vessel vasculitis

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PMID: 32441642 [PubMed]

Received: 19/11/2019
Accepted : 07/04/2020
In Press: 21/05/2020
Published: 21/05/2020

Abstract

OBJECTIVES:
This study researched the efficacy of rituximab (RTX) in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated hypertrophic pachymeningitis (HP).
METHODS:
Eight patients were identified by retrospective chart review from local registries at four hospitals in Japan. All patients met the Chapel Hill 2012 Consensus Conference definitions of ANCA-associated vasculitis and had disease complicated with HP. We assessed the dose of glucocorticoids, C-reactive protein (CRP) levels, Birmingham vasculitis activity score (BVAS) and contrast-enhanced magnetic resonance imaging (MRI) findings of HP before and after RTX administration.
RESULTS:
Three of eight patients were female. The median age was 68 years. No patients had HP at onset of vasculitis. Two patients had a relapse of HP before RTX administration. RTX was used as the initial treatment for HP in three patient. The daily dose of glucocorticoids, CRP levels and BVAS decreased from baseline to 6 months after RTX treatment in all patients. Evaluation of HP by contrast-enhanced MRI showed improvement in seven of eight cases. All of seven patients achieved sustained remission at 6 months after RTX treatment. No serious adverse events were observed in any patient.
CONCLUSIONS:
Our case series highlights the efficacy of RTX in patients with difficult-to-treat ANCA-associated HP. Future prospective studies are warranted to establish B-cell depletion therapy by RTX as a treatment option for ANCA-associated HP.

Rheumatology Article