Hypertrophic pachymeningitis associated with systemic lupus erythematosus: a case report
Authors: Mendes Larissa Braga , Souza Gabriel Pinheiro Martins de Almeida e , Moraes Marianna Pinheiro Moraes de , Pedroso José Luiz , Barsottini Orlando Graziani Povoas 
Published: July 2023

Introduction: Hypertrophic pachymeningitis is a chronic inflammatory process that results in focal or diffuse thickening of the dura mater. Nowadays, it’s an uncommon cause of neurological symptoms and has been mainly associated with infections, inflammatory diseases and carcinomatosis. This is a report of a single case based on retrospective analysis of the patient’s medical records. Case report: A 48-year-old woman was admitted in the emergency department with a two-month history of severe headaches that worsened with neck flexion and four episodes of focal to bilateral tonic-clonic seizures. In addition to that, she also complained of intermittent gritty sensation and redness in both eyes and arthritis of the hand and wrist joints. Her past medical history included systemic lupus erythematosus, bilateral lower limb deep venous thrombosis and cerebral venous thrombosis (CVT). She was on anticoagulant therapy with rivaroxaban 20 mg daily at the time. On admission, patient presented with bilateral conjunctival hyperemia and slit-lamp examination showed mild anterior chamber flare reaction (grade of 2+). Neurologic examination revealed impaired attention and nuchal rigidity. Brain magnetic resonance imaging was performed and showed linear dural thickening and diffuse pachymeningeal post-contrast enhancement over cerebral convexities, falx cerebri and cerebellar tentorium, with hyperintense signal on T2- weighted sequence in the subcortical region of the right frontal lobes, which might have resulted from obstruction of venous reflux caused by previous CVT. Brain venogram demonstrated partial recanalization of superior sagittal sinus. Lumbar puncture revealed a clear cerebrospinal fluid (CSF) with 12 cells/ mm3 (82% lymphocytes, 13% monocytes, 5% plasmocytes), total protein of 46 mg/dL and glucose of 58 mg/dL. Search for infectious agents by microscopy, culture and molecular testing resulted negative. Systemic investigation was negative for infections or metabolic dysfunction, but it revealed elevated inflammatory markers and hypocomplementemia. Her panel of autoantibodies revealed an ANA titer of 1:640 with a nuclear homogeneous pattern and was negative for the others (anti-dsDNA, anti-Sm, anti-RO, anti-LA, anti-RNP, RF). The patient was then treated with intravenous methylprednisolone 1 g daily for five days and followed by maintenance treatment with oral prednisone 60 mg daily. She showed good improvement of symptoms, as well as a significant reduction of CSF leucocytes and protein content. Unfortunately, it was not possible to obtain a meningeal biopsy at the time. The patient was finally discharged from the hospital and, given the rare association between pachymeningitis and SLE, is expected to return for a follow-up consultation with neurology for additional investigation. Conclusion: Although hypertrophic pachymeningitis has been related with some autoimmune disorders, it is an extremely rare form of nervous system manifestation in SLE. Despite the plausible relationship between the patient’s SLE disease activity and hypertrophic pachymeningitis, further investigation is still required to rule out other etiologies. Nevertheless, we hope our report will raise awareness of hypertrophic pachymeningitis as a possible differential diagnosis in SLE patients with new-onset headache with nuchal rigidity.