Hypertrophic Pachymeningitis as an Early Manifestation of Relapsing Polychondritis: Case Report and Review of the Literature

Ushiyama, Satoru; Kinoshita, Tomomi; Shimojima, Yasuhiro; Ohashi, Nobuhiko; Kishida, Dai; Miyazaki, Daigo; Nakamura, Katsuya; Sekijima, Yoshiki; Ikeda, Shu‐ichi

doi:10.1159/000450850

Cited by 12 publications

(9 citation statements)

References 14 publications

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“…Pachymeningitis has been reported in other cases of RP, but not in those cases that subsequently underwent cochlear implantation. 13 This case highlights that relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function, and hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.…”

Section: Discussionmentioning

confidence: 88%

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Akdoğan

Exilus

Ward

et al. 2021

Ann Otol Rhinol Laryngol

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Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods: Case report and literature review. Results: A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. Conclusions: Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.

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Section: Discussionmentioning

confidence: 88%

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Akdoğan

Exilus

Ward

et al. 2021

Ann Otol Rhinol Laryngol

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“…8 Of the 5 cases of pachymeningitis associated with RP indexed by PubMed, 3 were ANCA positive with PR3 specificity, suggesting that those cases may have been due to ANCA-associated vasculitis, which can also cause chondritis (table ). [9][10][11] The majority of previously reported cases of pachymeningitis associated with RP and/or IBD have had a relapsing course. Moreover, in this case, the pachymeningitis developed while the patient was on methotrexate.…”

Section: Discussionmentioning

confidence: 99%

Nodular Pachymeningitis Associated With Relapsing Polychondritis and Crohn Disease Responsive to Adalimumab and Prednisone

Hutto

Maher

Miloslavsky

et al. 2021

Neurol Neuroimmunol Neuroinflamm

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ObjectivesTo review the previous literature on the associations of pachymeningitis with Crohn disease (CD) and relapsing polychondritis (RP) and to describe a new case occurring in association with both in addition to highlighting its positive response to steroid and adalimumab treatment.MethodsWe review the patient's clinical presentation, diagnostic workup (serum and CSF testing), and MRI findings in detail and chronicle the response of the pachymeningitis to intensive immunotherapy. We contrast this case against previous reports of pachymeningitis occurring in association with RP and inflammatory bowel disease that were found on PubMed.ResultsOnly 2 cases of ulcerative colitis and 5 cases of RP were found in association with pachymeningitis; there were no cases in association with CD. Our patient presented with symptoms isolated to a steroid-responsive headache in the setting of normal neurologic and rheumatologic examinations. Her preceding history was notable for long-standing CD and increasingly active symptoms referable to RP. Focal nodular pachymeningitis was seen overlying the left hemisphere on brain MRI. An extensive serum and CSF workup and body fluorodeoxyglucose-PET scan failed to identify an alternative etiology beyond her underlying autoimmune inflammatory disorders. After adding prednisone and adalimumab to her preexisting treatment of methotrexate, she responded dramatically both clinically and radiographically.ConclusionsAlthough exceptionally rare, pachymeningitis may occur as a neuroinflammatory complication of CD and RP.

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“…The clinical presentation is acute, with stroke-like events and seizures 31,32,33 . There are reported cases of systemic lupus erythematosus, giant-cell arteritis, relapsing polychondritis, Behçet syndrome, and Sjögren syndrome presenting with HP 34,35,36,37,38 .…”

Section: Other Inflammatory Causesmentioning

confidence: 99%

A clinical approach to hypertrophic pachymeningitis

Abrantes

Moraes

Filho

et al. 2020

Arq. Neuro-Psiquiatr.

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Importance: Hypertrophic pachymeningitis (HP) is a non-usual manifestation of rheumatologic, infectious, and neoplastic diseases. Etiological diagnosis is a challenge, but when made promptly it creates a window of opportunity for treatment, with the possibility of a total reversal of symptoms. Observations: HP is an inflammatory process of the dura mater that can occur as a manifestation of sarcoidosis, granulomatosis with polyangiitis, and IgG4-related disease. The HP case evaluation is extensive and includes central nervous system imaging, cerebrospinal fluid analysis, serology, rheumatologic tests, and systemic survey for other manifestations sites. After systemic investigation, meningeal biopsy might be necessary. Etiology guides HP treatment, and autoimmune disorders are treated with corticosteroids alone or associated with an immunosuppressor. Conclusion: HP is a manifestation of several diseases, and a precise etiological diagnosis is crucial because of the difference among treatments. An extensive investigation of patients with HP helps early diagnosis and correct treatment.

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Hypertrophic Pachymeningitis as an Early Manifestation of Relapsing Polychondritis: Case Report and Review of the Literature

Cited by 12 publications

References 14 publications

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Nodular Pachymeningitis Associated With Relapsing Polychondritis and Crohn Disease Responsive to Adalimumab and Prednisone

A clinical approach to hypertrophic pachymeningitis

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