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New research · Ophthalmology
Immunologic research · 19h
Systematic reviewImmunologic research · 2026

Antineutrophil cytoplasmic antibody-associated pachymeningitis: a systematic review of clinical features, diagnosis and treatment outcomes.

Graziella Aguiar Santos Faria, Paula Baleeiro Rodrigues Silva, Gabriela Abrahão Allioni … Guilherme Diogo Silva
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OphthalmologySystematic review

Nearly a quarter of patients with antineutrophil cytoplasmic antibody-associated pachymeningitis relapsed after treatment.

Antineutrophil cytoplasmic antibody-associated pachymeningitis: a systematic review of clinical features, diagnosis and treatment outcomes.

Graziella Aguiar Santos Faria … Guilherme Diogo Silva
Immunologic research · 2026
Background

Antineutrophil cytoplasmic antibody (antineutrophil cytoplasmic antibody)-associated pachymeningitis is a rare inflammatory disorder of the dura mater that may occur in isolation or as part of systemic vasculitis.

Purpose

We aimed to synthesize clinical presentation, investigations, and treatment outcomes to improve diagnosis and guide management.

Methods

We identified 230 patients from 177 reports, including 108 myeloperoxidase-antineutrophil cytoplasmic antibody-positive, 71 PR3-antineutrophil cytoplasmic antibody-positive, and 3 dual myeloperoxidase/PR3-positive cases; 46 antineutrophil cytoplasmic antibody-positive cases had unspecified antigen specificity, and 2 were ELISA-negative but antineutrophil cytoplasmic antibody IIF-positive.

n = 230 patients
23.5%
Results
23.5%
About 1 in 4 patients had disease relapse after treatment, at median 9-month follow-up.
n = 230 patients
More results

Median age was 60 years; 53% were male.

Systemic involvement was present in 89% of cases, primarily affecting the ear, nose, throat, lungs, orbits and kidneys.

Inflammatory markers were raised in > 90%, and cerebrospinal fluid pleocytosis in 50%.

More results

Rituximab (25%) was associated with lower rates of refractory disease.

“
Conclusion

Relapse and incomplete recovery remain common despite treatment, underscoring the need for early recognition, targeted immunotherapy, and long-term follow-up.

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