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Histopathology and Glandular Biopsies in Sjögren’s Syndrome

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Sjögren’s Syndrome

Abstract

The glandular inflammatory lesion in Sjögren’s syndrome (SS) is a distinctive but not pathognomonic chronic lymphocytic adenitis best characterized in salivary glands. The glands are readily accessible; decreased function gives rise to prominent clinical symptoms and signs; and the glands are affected in almost all patients. A labial salivary gland biopsy specimen can be very disease specific for SS if it is obtained through normal-appearing mucosa, includes ≥5 separate glands separated from their surrounding connective tissue, is interpreted after lobes or glands showing non-specific changes are excluded, demonstrates focal sialadenitis in all or most of the glands in the specimen, and has a focus score that provides a diagnostic threshold. Such glandular biopsies can also provide tissue diagnosis for conditions that can resemble SS clinically, particularly sarcoidosis and amyloidosis. The characteristic pathologic lesion is part of classification criteria for SS and probably provides the best single criterion in terms of its disease sensitivity and specificity, convenience, availability, and low risk. More recently, studies have found germinal center reactions in the glandular biopsies indicating a more severe disease phenotype.

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Authors and Affiliations

  1. Broegelmann Research Laboratory, The Gade Institute, University of Bergen, Bergen, Norway

    Roland Jonsson

  2. Department of Rheumatology, Haukeland University Hospital, Bergen, Norway

    Roland Jonsson

  3. Broegelmann Research Laboratory, Department of Immunology, The Gade Institute, University of Bergen, Bergen, Norway

    Kathrine Skarstein

  4. Institute of Medicine-Section for Rheumatology, University of Bergen, Bergen, Norway

    Malin V. Jonsson

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  1. Roland Jonsson
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Correspondence to Roland Jonsson .

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  1. Research Foundation, Rheumatology Clinic, Scripps Memorial Hospital &, Caminito Sinnecock 6565, La Jolla, 92037, California, USA

    Robert I. Fox

  2. Research Foundation, Rheumatology Clinic, Scripps Memorial Hospital &, Caminito Sinnecock 6565, La Jolla, 92037, California, USA

    Carla M. Fox

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Jonsson, R., Skarstein, K., Jonsson, M.V. (2011). Histopathology and Glandular Biopsies in Sjögren’s Syndrome. In: Fox, R., Fox, C. (eds) Sjögren’s Syndrome. Springer, New York, NY. https://doi.org/10.1007/978-1-60327-957-4_6

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