Pachymeningitis in Rheumatic Disease

Rachel Kneeland¹, John Berry ², Sydney Brandwein ² and Monika Starosta ², ¹Department of Internal Medicine, Advocate Lutheran General Hospital, Park Ridge, IL, ²Advocate Lutheran General Hospital, Park Ridge, IL

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Nervous system lupus, Neuroimaging, neurology and rheumatic disease, Rheumatoid arthritis (RA)

Session Information

Date: Tuesday, November 12, 2019

Title: Miscellanous Rheumatic & Inflammatory Disease Poster III: Autoimmune Conditions and Therapies

Session Type: Poster Session (Tuesday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Pachymeningitis is a rare complication of rheumatic disease. Patients present with hearing loss or other neurologic complications. Diagnosis is made by thickening of dura or leptomeninges on MRI-imaging or histopathologically with biopsy of the dura. The prevalence pachymeningitis in rheumatic disease is unknown, and there is no standard of care.

Methods: We retrospectively identified nine patients diagnosed with pachymeningitis based on clinical symptoms, MRI findings, and in three cases, dural biopsy. We assessed the rheumatologic diagnoses, presenting symptoms, treatment modalities, and outcomes.

Results: There were two males and seven females, with a mean age of 63 for males and 60 for females. Five patients had rheumatoid arthritis, two had sarcoidosis, one had undifferentiated connective tissue disease and Sjogren’s syndrome, and one had blastomycosis who developed rheumatoid arthritis one year later. The presenting symptoms were hearing loss (three), unilateral weakness (two), unilateral numbness (two), seizure (two). Additional symptoms included headache, confusion, memory loss, tinnitus, scleritis, optic neuritis, unilateral hemineglect, foot flaccidity, and gait disturbance. Diagnosis was confirmed in three patients with dural biopsy. MRI images were obtained on all patients. Six patients were treated solely with prednisone or pulse-dose methylprednisolone. Two patients were treated with anti-TNF agents and one patient with rituximab. Pre- and post-treatment MRI demonstrated resolution of leptomeningeal abnormality in two patients. Six out of nine patients reported resolution of symptoms with treatment. Three had persistent deficits, including hearing loss, visual impairment, and slight residual hemiplegia.

Conclusion: Rheumatoid arthritis was the most common rheumatological diagnosis, and hearing loss was the most common presenting symptom. Most patients had resolution of symptoms with prednisone, pulse methylprednisolone, anti-TNF agents, or rituximab. We hope this case series will assist clinicians with the diagnosis and management of pachymeningitis.

Table 1

Table 1. Age, sex, rheumatologic diagnosis -Dx-, presenting symptoms, outcomes and treatments of nine patients identified to have pachymeningitis based on clinical symptoms, magnetic resonance imaging -MRI- findings, and in three instances, dural biopsy. Rheumatologic diagnoses include rheumatoid arthritis -RA-, Sarcoidosis -SD-, Undifferentiated Connective Tissue Disease -UCTD-, Limited cutaneous Systemic Sclerosis -CREST-, Sjogren’s Disease -SJ-, Blastomycosis -BM-, Ulcerative Colitis -UC-. Lateralization of deficits abbreviated by L -Left- and R -Right-. Treatment of patients was typically with high dose -HD- prednisone defined as 50 to 60 mg daily, pulse dose -PD- methylprednisolone of 500 mg to 1000 mg daily for three to five days.

Figure 1

Figure 1. Magnetic resonance imaging -MRI- of the brain from individuals with pachymeningitis. -A- pachymeningitis with leptomeningeal enhancement overlying the occipital lobe -arrow- in a patient with RA presenting with optic neuritis; -B- Unilateral dural and leptomeningeal thickening and enhancement over superior R frontal lobe convexity and sulci extending into interhemispheric fissure in R medial frontal lobe in a patient with blastomycosis who developed focal seizure and L sided numbness who ultimately developed RA one year later; -C1, C2- Coronal and transverse plane images -respectively- demonstrating R cerebral dural, leptomeningeal enhancement seen in patient with RA presenting with acute confusion and L hemiparesis, L sided sensory loss, and L hemineglect; -D1, D2- Coronal and transverse plane images -respectively- demonstrating diffuse bilateral meningeal enhancement after gadolinium in a patient with RA presenting with R unilateral hearing loss, scleritis, and synovitis.

Disclosure: R. Kneeland, None; J. Berry, None; S. Brandwein, None; M. Starosta, None.

To cite this abstract in AMA style:

Kneeland R, Berry J, Brandwein S, Starosta M. Pachymeningitis in Rheumatic Disease [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/pachymeningitis-in-rheumatic-disease/. Accessed .

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