Autoimmune neutropaenia complicating Sjögren's Syndrome: haematological and clinical improvement with granulocyte colony stimulating factor

Connective tissue diseases are associated with granulocyte-specific autoantibodies and autoimmune neutropaenia. Reduced levels of circulating neutrophils may predispose to recurrent infections, particularly of the respiratory tract, although severe and opportunistic infections have also been reported. There is only one previous description of the use of granulocyte colony stimulating factor (GCSF) in Sjögren's Syndrome complicated by autoimmune neutropaenia, reporting poor response and tolerability. We present here the successful use of GCSF in a 42-year-old woman with Sjögren's Syndrome, autoimmune neutropaenia and chronic mastoiditis.