A case report described the first case of a patient with Crohn’s disease (CD) and recurrent polychondritis (RP) who presented with pachymeningitis, according to study results published in Neurology Neuroimmunology and Neuroinflammation.
Massachusetts General Hospital study researchers reviewed the clinical presentation of a patient with pachymeningitis. They reviewed literature databases for previous reports of pachymeningitis and compared the results of historical data with their current observations.
A 48-year-old woman with a history of celiac disease and RP was referred for evaluation for 9 months of new onset headache on the left side of the head associated with scalp tenderness. The patient was diagnosed with CD with minimal abdominal symptoms 11 years ago for which she was taking balsalacide. She was diagnosed with RP 6 years ago with symptoms in the septum, scleritis and episcleritis, and chondritis. During the year preceding the headache, RP symptoms progressed gradually and the patient was given methotrexate as maintenance therapy around the onset of the headache.
On magnetic resonance imaging, the patient presented with abnormal smooth dural thickening and left cerebral hemisphere enhancement with focal nodular thickening and heterogeneous enhancement over the left frontal and temporal operculum. The area of the pachymeningeal thickening corresponded to restricted diffusion.
No evidence of neurological symptoms such as seizures, motor symptoms or changes in mental function was reported. General, neurological, and rheumatological evaluations were normal.
All serum laboratory characteristics examined were normal, with the exception of C-reactive protein (42.1 mg / L) and erythrocyte sedimentation rate (48 mm / h), which were increased. Examination of the cerebrospinal fluid showed abnormal white blood cell counts (5 white blood cells / mm3), protein (21 mg / dL), glucose (72 mg / dL), and opening pressure (27 mm H2O).
Health care providers gave the patient prednisone (60 mg / day, reduced to 10 mg / day), adalimumab for 2 months and advised her to keep using the methotrexate that was already present. After 2 months, the patient’s headache had subsided and the smooth pachymeningeal thickening and reinforcement subsided.
The study’s investigators found 5 cases of RP-related pachymeningitis in their literature review. The majority of previously reported cases relapsed.
In this case study, RP was likely the driving force behind pachymeningitis, however, since this patient had multiple autoimmune diseases, pachymeningitis may have been an unrelated comorbidity.
Based on their findings, the study’s investigators concluded that “pachymeningitis, although extremely rare, can occur as a neuroinflammatory complication of CD and RP”.
Hutto SK, Maher MD, Miloslavsky EM, Venna N. Nodular pachymeningitis related to recurrent polychondritis and Crohn’s disease in response to adalimumab and prednisone. Neurol Neuroimmunol Neuroinflamm. 2021; 8 (5): e1022. doi: 10.1212 / NXI.0000000000001022