Combination of Hypertrophic Pachymeningitis, PR3-ANCA-positive Vasculitis, and Relapsing Polychondritis

Cañas, Carlos A.; Díaz-Martínez, Juan Carlos; Tobón, Gabriel J.

doi:10.3899/jrheum.101238

Cited by 12 publications

(6 citation statements)

References 7 publications

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“…3,4 All the three previously reported RP patients complicated by hypertrophic pachymeningitis showed ANCA-positive vasculitis, with an implication that ANCA-related vasculitis might be the cause of hypertrophic pachymeningitis in these patients. 4 We also excluded recognizable autoimmune diseases and paraneoplastic syndrome according to the clinical presentation and laboratory examinations. A post-mortem pathological study of RP reported diffuse vasculitis of the brain, indicating that it extended to the intracranial dura mater.…”

Section: Discussionmentioning

confidence: 97%

“…Although the present patient was ANCA‐negative, hypertrophic pachymeningitis is frequently associated with systemic vasculitis, such as Wegener's granulomatosis, particularly in ANCA‐positive patients, and immunoglobulin G4‐related disease . All the three previously reported RP patients complicated by hypertrophic pachymeningitis showed ANCA‐positive vasculitis, with an implication that ANCA‐related vasculitis might be the cause of hypertrophic pachymeningitis in these patients .…”

Section: Discussionmentioning

confidence: 99%

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Hypertrophic pachymeningitis and encephalitis in a patient with relapsing polychondritis

Nakamura

Sugaya

Nakata

et al. 2014

Neurology & Clinical Neurosc

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Relapsing polychondritis is a rare autoimmune multisystem disease characterized by the inflammation of cartilaginous tissues. Neurological complications with relapsing polychondritis are extremely rare. We report an 81‐year‐old woman with relapsing polychondritis who subsequently developed encephalitis and cognitive impairment. Brain magnetic resonance imaging showed slightly high signal intensity in the bilateral frontal dura mater using fluid‐attenuated inversion recovery. T1‐weighted images with contrast enhancement showed abnormal enhancement of the dura mater, indicating hypertrophic pachymeningitis. To our knowledge, this is the first case report of relapsing polychondritis complicated by both hypertrophic pachymeningitis and encephalitis in the absence of other recognizable autoimmune diseases.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Hypertrophic pachymeningitis and encephalitis in a patient with relapsing polychondritis

Nakamura

Sugaya

Nakata

et al. 2014

Neurology & Clinical Neurosc

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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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“…HP is an inflammatory disorder that occurs secondary to intracranial infections, neoplastic diseases, and some autoimmune disorders such as ANCA-associated vasculitis (AAV), sarcoidosis, and IgG4-related disease [7]. To our knowledge, only four cases of HP related to RP have been reported in two original articles [8, 9] (table 1). However, AAV was also demonstrated in three of those cases, suggesting that AAV might affect the occurrence of HP; incidentally, AAV is the most common cause of HP [7].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2016

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Neurological involvement in relapsing polychondritis (RP) is relatively rare. We describe the case of an 80-year-old man who presented with hypertrophic pachymeningitis (HP) together with arthritis as the first manifestation of RP. Auricular chondritis, which subsequently determined the diagnosis of RP, occurred a few weeks after the detection of HP. The neurological symptoms, as well as arthritis, were promptly improved by treatment with corticosteroids. It is generally difficult to diagnose RP in the absence of typical cartilaginous involvement; however, the present case suggests that HP may occur as an early clinical manifestation of RP.

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Combination of Hypertrophic Pachymeningitis, PR3-ANCA-positive Vasculitis, and Relapsing Polychondritis

Cited by 12 publications

References 7 publications

Hypertrophic pachymeningitis and encephalitis in a patient with relapsing polychondritis

Hypertrophic pachymeningitis and encephalitis in a patient with relapsing polychondritis

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

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

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