P047 Enteroviral infection in autoimmune rheumatic diseases treated with anti-CD20 monoclonal antibodies

This case series describes two cases of CNS enteroviral infection in immunosuppressed patients with autoimmune rheumatic diseases (AIRDs). Enteroviral meningoencephalitis, a typically mild and self-limiting condition in immunocompetent individuals, can present as a severe, protracted illness with complex neurological manifestations in these patients. Immunosuppressants, particularly Anti-CD20 monoclonal antibodies (mAbs), can heighten the risk of complex enteroviral infection.

CASE 1 A 41-year-old female presented with an acute confusional state, headache, nausea and incoherent speech. She had background of granulomatosis with polyangiitis diagnosed 10 years previously and receiving mycophenolate and rituximab (last administered 4 months prior). On examination, she had a flat affect, reduced verbal fluency, brisk reflexes(3+) but without overt of meningism. Laboratory acute phase markers and neuroimaging investigations were normal. Electroencephalogram showed left temporal sharp waves, implying cortical irritation. Cerebrospinal fluid (CSF) revealed raised white cell count (WCC) 580 (0-5) with 99% lymphocytes and hyperproteinorrachia (>2g/L).

CASE 2 A 27-year-old lady with systemic lupus erythematosus diagnosed in her teens presented with new neurological symptoms of slurred speech and vertigo. Neurological examination revealed right-sided nystagmus and ataxic gait. Two weeks preceding, she had had sequential admissions due to cough, nausea, vomiting, dizziness and persistent headache. She was diagnosed with right-sided vestibular neuritis at that time with evidence of enhancement in the posterosuperior aspect of the distal internal auditory meatus (IAM) on MRI brain and IAM. Due to active lupus nephritis, she was receiving multiple immunosuppressants including mycophenolate and with the recent addition of obinutuzumab 2 months prior. Given her progressive symptoms and immunosuppressed state, lumbar puncture was performed. CSF revealed elevated WCC of 44, predominantly lymphocytes and raised protein (800mg/dL).

In both cases, CSF PCR was positive for enterovirus. Patients were diagnosed with enteroviral meningoencephalitis. They were treated with intravenous Immunoglobulin (IVIg). Although more often used in autoimmune encephalitis, IVIg has putative benefits iin viral encephalitis; potentially by increasing viral clearance by antibody-dependent neutralization and via a direct immunomodulatory action on CSF hyperinflammation and was used due to the lack of targeted antiviral treatments for enteroviruses.

In the first case, the patient recovered after weeks but has residual headaches. In the second case, patient recovery was gradual with persistent speech impairment over months.

Enteroviruses are common causes of viral meningitis, but they can present atypically and more severely in immunocompromised individuals and should be considered in those patients presenting with neurological symptoms, even when typical signs of meningitis are absent. These cases demonstrate that enteroviral infections can lead to prolonged and debilitating illnesses in these patients. Diagnosis relies on CSF PCR testing, which is highly sensitive and specific for detecting enteroviruses. It remains challenging to find a delicate balance in managing immunosuppression to control autoimmune disease while mitigating the risk of opportunistic infections.

S. Tio: None. F. Zia: None. D. Costello: None. S. Moran: None. A. Jackson: None. G. Murphy: None.