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

Abstract

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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“…However, reports of direct CNS involvement in Crohn disease are rare and involve cerebral lesions with a single case of pachymeningitis reported in a patient with coexisting relapsing polychondritis. 10,11 This case also raises the potential association between malignancy and granulomatous inflammation. The term "sarcoidlike reaction" (SLR) is sometimes used in the literature to designate the presence of noncaseating granuloma pathologically indistinguishable from sarcoidosis but hypothesized to be triggered by an antigen, commonly in the setting of malignancy or medication.…”

“…While the patient did not have active gastrointestinal disease, it is recognized that extraintestinal manifestations often do not parallel intestinal disease; furthermore, the patient's long-term immunosuppression had been ceased. However, reports of direct CNS involvement in Crohn's disease are rare and involve cerebral lesions with a single case of pachymeningitis reported in a patient with co-existing relapsing polychondritis 10,11 .…”

“…However, reports of direct CNS involvement in Crohn disease are rare and involve cerebral lesions with a single case of pachymeningitis reported in a patient with coexisting relapsing polychondritis. 10,11…”