Severe lupus vasculitic neuropathy

A 31-year-old woman presented with generalised pain and weakness due to a severe axonal sensorimotor neuropathy, rapidly worsening over 2 weeks. For 6 months she had experienced transient joint symptoms, rash and hair loss. A vasculitic process was considered. Blood tests taken following intravenous immunoglobulin (IVIG) (given for suspected Guillain-Barré syndrome) noted strongly positive lupus and antiphospholipid antibody titres. She was diagnosed with severe multisystem lupus vasculitis involving the central and peripheral nervous system and started on cyclophosphamide and prednisolone, with notable improvement. We outline challenges faced in this patient's care, including making the diagnosis, interpreting autoantibody serology following IVIG treatment and identifying suitable immunosuppression regimens in a limited evidence base. It is important to be aware that peripheral neuropathy is an under-recognised presenting manifestation of neurological lupus.