• Internal medicine · Nov 2022

    Case Reports

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

    • Katsuyuki Tomita, Shinnosuke Tokugawa, Genki Inui, Shotaro Moriyasu, Tomoyuki Ikeuchi, Hirokazu Touge, and Fuminari Tajima.
    • Department of Respiratory Medicine, National Hospital Organization Yonago Medical Center, Japan.
    • Intern. Med. 2022 Nov 1; 61 (21): 328732913287-3291.

    AbstractPrimary 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. A patient with pSS concomitant with axonal SMP might show severe neutropenia as aggressive autoimmune disease. Neutropenia can be treated with systemic glucocorticoids based on the assessment of NAIgG on the neutrophil surface.

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