Recurrent Aseptic Meningitis Can Be First Symptom of Sjögren’s Syndrome, Case Report Suggests

Doctors should suspect primary Sjögren’s syndrome when examining patients, especially young women, with recurrent aseptic meningitis — a form of inflammation of the meninges, a membrane protecting the brain and spinal cord, that is not caused by bacteria, a case report suggests.

The study, “Recurrent aseptic meningitis: A rare clinical presentation of Sjogren’s syndrome,” was published in the Journal of the Pakistan Medical Association.

Sjogren’s syndrome is an autoimmune disease mostly characterized by dry eyes and mouth, joint pain, and fatigue. Complications of the central nervous system, like aseptic meningitis and meningoencephalitis — an inflammation of the meninges and brain — are known to affect about 20% of patients. Rarely a first symptom of Sjogren’s, they pose a real diagnostic challenge when they are.

Researchers in Pakistan report the case of a 19-year-old woman with recurrent aseptic meningitis as the initial symptom of Sjogren’s syndrome.

The woman had a first episode of aseptic meningitis around age 12, and was hospitalized with fever, headache, and vomiting for four days. Analysis of her cerebrospinal fluid (CSF, the fluid surrounding the brain and spinal cord, usually collected by spinal tap) indicated meningitis not caused by bacteria, which responded well to treatment.

Six years later, however, the woman was readmitted with increasing headache, light sensitivity, vomiting, shoulder pain, neck pain, and stiffness. CSF analysis was again consistent with non-bacterial meningitis, but this time she only responded to treatment for one month.

After that month, she was again readmitted to the hospital with the same symptoms. Researchers examined the patient’s clinical history, and found that she had been experiencing dryness of the eyes and mouth for the last three years and fatigue for the last six months. She also had a history of joint pain.

Blood analysis showed she was positive for anti-nuclear antibodies, and SSA/Ro antibodies, leading to a diagnosis of Sjögren’s syndrome with recurrent aseptic meningitis.

The patient received dexamethasone for two days followed by treatment with oral prednisolone and hydroxychloroquine. She responded well to the treatment and was symptom free after two weeks, which was maintained for at least 11 months, the time of her last follow-up.

“We suggest that SS [Sjogren’s syndrome] should be considered in the differential diagnosis of recurrent aseptic meningitis, especially in a young female patient with recurrent symptoms,” the researchers concluded.