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

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.

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

“…We found that the most common type of HP was idiopathic HP, followed by AAV-related HP, tuberculous-associated HP, viral-related HP, and bacterial-related HP. No sex preference was identified in our study, which was not consistent with previous works (2)(3)(4). The onset of the first symptoms mainly occurred at an age of 50 ± 12 years, which was earlier than the patients in the US and Japan (2,5).…”

“…In a recently published article, the HP incidence was significantly higher in patients with GPA than in those with MPA (60.2 vs. 3.3 persons per 1,000 person-years, respectively) ( 46 ). The reasons why AAV-related disorders affect the CNS may include the following: ( 1 ) granulomatosis tissue extends directly to the intracranial nervous system from adjacent lesions in the orbit or the paranasal cavity, ( 2 ) granulomatosis tissue transfers to the intracranial nervous system from the respiratory tract, and ( 3 ) vasculitis affects intracranial vessels ( 29 ).…”

“…Such a low prevalence contributes to the fact that most published articles are case reports, which has made it difficult to draw a complete picture of the disease. Furthermore, most HP cases have been reported in the Caucasian population; only a few studies of HP, with relatively small sample sizes, are available in China (3)(4)(5). In this study, we retrospectively investigated 48 patients diagnosed with HP in our neurology department.…”

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

“…We found that the most common type of HP was idiopathic HP, followed by AAV-related HP, tuberculous-associated HP, viral-related HP and bacterial-related HP. No sex preference was identified in our study, which was not consistent with previous works [2][3][4]. The onset of the first symptoms mainly occurred at an age of 50±12 years, which was earlier than the patients in the US and Japan [2,5].…”

“…Such a low prevalence contributes to the fact that most published articles are case reports, which has made it difficult to draw a complete picture of the disease. Furthermore, most HP cases have been reported in the Caucasian population; only a few studies of HP, with relatively small sample sizes, are available in China [3][4][5]. In this study, we retrospectively investigated 48 patients diagnosed with HP in our neurology department.…”