Anti-neutrophil cytoplasmic antibody-associated hypertrophic cranial pachymeningitis and otitis media: a review of literature.

BACKGROUND AND OBJECTIVE: It has been recognized that anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides may lead to hypertrophic pachymeningitis (HP) or intractable otitis media (OM). To our knowledge, few cases of coexistent ANCA-related HP and OM have been described previously. To increase awareness of this disease, we reviewed the literature describing patients with HP and intractable OM in a population with AAV to guide clinical decision making for otolaryngologists.

METHODS:  PubMed was searched with the following terms: ANCA-associated vasculitis, otitis media, and hypertrophic pachymeningitis. Only patients with concomitant AAV, OM and HP were considered and included in this review.

RESULTS: A total of 243 articles were reviewed, and of these, 6 met inclusion criteria. Headache, cranial polyneuropathy, and intractable OM with effusion or granulation were common. Serum MPO-ANCA positivity was most common in Asian patients. Almost all patients had dural mater thickening on gadolinium-enhanced magnetic resonance imaging of the brain. Corticosteroids plus an immunosuppressant was more effective and most patients had improved hearing after treatment, but approximately 50% of subjects had disease relapse.

CONCLUSION: In this review, we summarized the current knowledge on the clinical features, diagnosis, treatment, and pathogenesis of this disease. We should carefully detect the potential cases of ANCA-related HP and OM in patients with intractable OM, HP, or AAV, and make the optimal treatment plan to avoid long-term neurological complications and irreversible hearing loss. Furthermore, due to an increased possibility of relapse, close follow-up, including a hearing test, ANCA titers, imaging examination, and detection of toxic and side effects of immunosuppressive therapy, are necessary.