Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS)

Abstract

Background This study investigated the characteristics of hypertrophic pachymeningitis (HP) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), using information from a multicenter study in Japan. Methods We analyzed the clinical information of 663 Asian patients with AAV (total AAV), including 558 patients with newly diagnosed AAV and 105 with relapsed AAV. Clinical findings were compared between patients with and without HP. To elucidate the relevant manifestations for HP development, multivariable logistic regression analyses were additionally performed. Results Of the patients with AAV (mean age, 70.2 ± 13.5 years), HP was noted in 30 (4.52%), including 20 (3.58%) with newly diagnosed AAV and 10 (9.52%) with relapsed AAV. Granulomatosis with polyangiitis (GPA) was classified in 50% of patients with HP. A higher prevalence of GPA was significantly observed in patients with HP than in those without HP in total AAV and newly diagnosed AAV (p < 0.001). In newly diagnosed AAV, serum proteinase 3 (PR3)-ANCA positivity was significantly higher in patients with HP than in those without HP (p = 0.030). Patients with HP significantly had ear, nose, and throat (ENT) (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.03–2.14, p = 0.033) and mucous membrane/eye manifestations (OR 5.99, 95% CI 2.59–13.86, p < 0.0001) in total AAV. Moreover, they significantly had conductive hearing loss (OR 11.6, 95% CI 4.51–29.57, p < 0.0001) and sudden visual loss (OR 20.9, 95% CI 5.24–85.03, p < 0.0001). Conclusion GPA was predominantly observed in patients with HP. Furthermore, in newly diagnosed AAV, patients with HP showed significantly higher PR3-ANCA positivity than those without HP. The ear and eye manifestations may be implicated in HP development.

Clinical characteristics of hypertrophic cranial pachymeningitis in granulomatosis with polyangiitis: a retrospective single-center study in China

“…In the model, PR3-ANCA negativity, conductive or sensorineural hearing loss, and decreased or sudden visual loss were identified as statistically significant variables, consistent with previous studies indicating that conductive hearing loss and sudden visual loss were significant factors in patients with HP related to AAV [ 8 ]. Therefore, these three covariates were selected to ensure statistical robustness, especially given the small sample size, while balancing clinical and statistical considerations.…”

“…Granulation or effusion in the middle ear spreading to the dura mater is thought to be a probable cause of HCP [ 15 ]. Additionally, Shimojima et al demonstrated that sudden visual loss and conductive hearing loss were significantly recognized in patients with AAV-associated HP, suggesting that inflammatory lesions proximal to CNS may play a pivotal role in the pathogenesis of AAV-associated HP [ 8 ]. Conversely, patients with GPA-related HCP were less likely to have pulmonary and renal involvement [ 2 ], which can potentially lead to misdiagnosis as localized GPA without identifying life-threatening organ dysfunction at the first diagnosis.…”

Rituximab for treatment of pachymeningitis in granulomatosis with polyangiitis

“…Our case is unique because the patient developed pachymeningitis after a prior cryptococcal infection, which has not been previously reported in cases of HP due to ANCA vasculitis 7–9. Considering the patient’s history of pulmonary cryptococcosis and the finding of HP on MRI, cryptococcal meningitis was considered as a potential diagnosis.…”

“…Hypertrophic pachymeningitis in GPA (GPA-HP) is a rare granulomatous manifestation presenting with headache, visual symptoms and cranial nerve involvement 7. GPA-HP rarely manifests as the first presenting symptom and usually arises during relapses with or without other organ involvement.…”

“…Our case also demonstrates the effectiveness of RTX in the treatment of HP caused by GPA with a favourable clinical, radiological and serological response. CYC and other immunosuppressive have been shown to be effective in treating GPA-HP in multiple case series and case reports but results are inconsistent 7–10. Similarly, the literature review on the efficiency of RTX in GPA-HP yielded only one case series of eight patients by Kobayashi et al and 11 other case reports 11.…”