Recurrent Aseptic Basal Meningitis as the First Clinical Manifestation of a Sjogren Syndrome in a Patient with an Overlap Syndrome with Familial Mediterranean Fever

Objective: In patients suffering from Sjogren Syndrome (SjS) central nervous system involvement, such as aseptic meningitis, commonly occurs. MRI scans of the brain usually show T2-hyperintensities and/or contrast-enhanced lesions. However, aseptic meningitis may also occur in patients with Familial Mediterranean Fever (FMF). Typical clinical symptoms are recurrent attacks of fever and polyserositis. Of note, in patients with FMF the symptoms usually disappear during a colchicine therapy.

Here, we present a patient for the first time with an overlap syndrome of SjS and FMF suffering from recurrent aseptic meningitis which precedes the diagnosis of SjS and shows imaging signs of a basal meningitis.

Case: A female patient suffered from biopsy-proven SjS and genetically diagnosed FMF. Over two years she presented with recurrent attacks of aseptic meningitis, while lacking symptoms of polyserositis indicating FMF not being causative. These attacks preceded the diagnosis of SjS. Cranial MRI revealed basal contrast enhancement of the cerebellum.

Meningitis persisted during colchicine therapy but terminated after initiating immunosuppressive treatment.

Conclusion: Recurrent aseptic meningitis may occur as the first clinical manifestation of SjS. In a basal and aseptic meningitis SjS should be considered in the differential diagnosis.