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Pathogenesis and Management of Primary Sjögren’s Syndrome

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Summary

Primary Sjögren’s syndrome is a chronic immuno-inflammatory connective tissue disorder characterised by lymphocytic exocrinopathy involving lacrimal and salivary glands, and causing dryness of ocular and oral mucosae. However, all exocrine glands may be affected by the autoimmune damage. Nonexocrine involvement is frequent and comprises inflammatory and noninflammatory vasculopathy, mediator-induced manifestations, and autoimmune endocrinopathies. Multiple factors are probably involved in the aetiopathogenesis, which besides immuno-inflammation is characterised by lymphoproliferation, sometimes leading to non-Hodgkin’s lymphoma. Blood levels of soluble immuno-inflammatory products are correlated with the extent and activity of exocrine and/or nonexocrine involvement, and may therefore be used as disease activity markers. Histopathological findings are important for the grading of the lymphocytic exocrinopathy (focus scoring) and for diagnosing vasculitis and lymphoma.

Systemic immunomodulating therapy is generally avoided because primary Sjögren’s syndrome usually has a benign course, but it may be necessary in severe or progressive monoclonal B lymphocyte disease, internal organ exocrine disease or vasculitis. Monitoring of keratoconjunctivitis sicca and xerostomia requires regular examination of secretory capacity and clinical evaluation of mucosal membranes. Bromhexine and pilocarpine may be used to increase lacrimal and salivary gland output, respectively. Tear and saliva substitutes are local treatment measures that help to prevent mucosal complications. Frequent dental control is mandatory for the prevention and management of sequelae to the teeth.

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Authors and Affiliations

  1. Department of Medicine TTA and Department of Ophthalmology E, Rigshospitalet, National University Hospital, Copenhagen, Denmark

    Peter Oxholm

  2. Eye Pathology Institute, University of Copenhagen, Copenhagen, Denmark

    Jan U. Prause

  3. Hillerød Sjögren-Center and Department of Oral Medicine and Oral Surgery, Central Hospital, Hillerød, Denmark

    Morten Schiødt

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  1. Peter Oxholm
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  2. Jan U. Prause
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  3. Morten Schiødt
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Oxholm, P., Prause, J.U. & Schiødt, M. Pathogenesis and Management of Primary Sjögren’s Syndrome. Clin Immunother 2, 421–429 (1994). https://doi.org/10.1007/BF03259043

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