Abstract
Background and objectiveIt 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.ResultsA 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.ConclusionIn 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.
Highlights
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels associated with anti-neutrophil cytoplasmic antibody (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3)
We identified 243 articles but reviewed 20 studies describing hypertrophic pachymeningitis (HP) associated with otitis media (OM) (Table 1) [8–27], and of these, six met inclusion criteria (Fig. 1)
Patients were diagnosed with ANCA-related HP or OMAVV if serum MPO–ANCA and/or PR3-ANCA titers were elevated or if ANCA-related disease co-occurred with HP or OM
Summary
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels associated with ANCA specific for myeloperoxidase (MPO) or proteinase 3 (PR3). Some patients may require treatment for hearing loss, headache, with or without cranial nerve paralysis Such cases are misdiagnosed, so better criteria are needed for early diagnosis and to avoid long-term neurological complications and irreversible hearing loss. It has been recognized that anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides may lead to hypertrophic pachymeningitis (HP) or intractable otitis media (OM). 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. 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. 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
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