Granulocyte-colony-stimulating Factor-resistant Neutropenia and Polyneuropathy Presenting as Severe Complications of Sjögren's Syndrome.

Primary Sjögren's syndrome (pSS) has multi-dimensional manifestations, including neutropenia and polyneuropathy. We herein report a 76-year-old woman with pSS initially presenting as severe granulocyte-colony-stimulating factor (G-CSF)-refractory neutropenia and axonal sensorimotor polyneuropathies (SMP). Systemic glucocorticoid administration had reduced neutrophil-associated immunoglobulin G (NAIgG) on the neutrophil surface as detected using flow cytometry, resulting in the development of neutropenia.