A case report described the first instance of a patient with Crohn disease (CD) and relapsing polychondritis (RP) presenting with pachymeningitis, according to study findings published in Neurology Neuroimmunology and Neuroinflammation.
Study researchers from Massachusetts General Hospital reviewed a clinical presentation of a patient with pachymeningitis. They reviewed literature databases for previous reports of pachymeningitis and contrasted findings of historical data with their current observations.
A woman aged 48 years with a history of CD and RP was referred for evaluation due to 9 months of new-onset headache on the left side of her head coupled with scalp sensitivity. The patient received a CD diagnosis 11 years previously with minimal abdominal symptoms for which she was taking balsalazide. She had been diagnosed with RP 6 years previously with symptoms occurring in the septum, scleritis and episcleritis, and chondritis. During the year preceding the headaches, RP symptoms gradually progressed, for which the patient was given maintenance methotrexate around headache onset.
At magnetic resonance imaging, the patient presented with abnormal smooth dural thickening and enhancement of the left cerebral hemisphere featuring focal nodular thickening and heterogeneous enhancement over the left frontal and temporal operculum. The region of pachymeningeal thickening corresponded with restricted diffusion.
No evidence of neurologic symptoms such as seizures, motor symptoms, or changes in mental functions were reported. General, neurologic, and rheumatologic evaluations were normal.
All assessed serum laboratory features were normal except for C-reactive protein (42.1 mg/L) and erythrocyte sedimentation rate (48 mm/h), which were elevated. Cerebrospinal fluid assessment indicated abnormal white blood cell count (5 white blood cells/mm3), protein (21 mg/dL), glucose (72 mg/dL), and opening pressure (27 mm H2O).
Healthcare providers administered the patient prednisone (60 mg/day tapered to 10 mg/day), adalimumab for 2 months, and advised her to continue using pre-existing methotrexate. At 2 months, the patient’s headaches had subsided and the smooth pachymeningeal thickening and enhancement had been resolved.
The study investigators found 5 cases of pachymeningitis associated with RP in their literature search. The majority of previously reported cases were relapsing.
For this case study, the RP was likely the driving force of pachymeningitis, however, as this patient had multiple autoimmune diseases, the pachymeningitis may have been an unrelated comorbid disease.
Based on their findings, the study investigators concluded that, “although exceptionally rare, pachymeningitis may occur as a neuroinflammatory complication of CD and RP.”
Hutto SK, Maher MD, Miloslavsky EM, Venna N. Nodular Pachymeningitis Associated With Relapsing Polychondritis and Crohn Disease Responsive to Adalimumab and Prednisone. Neurol Neuroimmunol Neuroinflamm. 2021;8(5):e1022. doi:10.1212/NXI.0000000000001022