Antineutrophil cytoplasmic antibody-associated pachymeningitis: a systematic review of clinical features…

Systematic reviewImmunologic research· May 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

OphthalmologySystematic reviewPrognosisQ3IF 2.7

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 · May 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.

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Conclusion

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

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

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

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