Severe headache in primary Sjögren's syndrome treated with intrathecal rituximab.

A severe and persistent migrainous headache in a patient with primary Sjøgren's syndrome unresponsive to treatment with immunosuppressive drugs, triptans, opioids, and NSAIDs, responded successfully to intrathecal B-cell depletion with rituximab. We hypothesize that brain-resident autoreactive B cells were involved in headache pathogenesis and were eliminated by this procedure.