Primary Sjögren's syndrome: the challenge for classification of disease manifestations


  • The study was supported by the Danish Rheumatism Association. All members of the Copenhagen Study Group for Sjögren's syndrome are sincerely thanked for constructive discussions during preparation of the manuscript.

Dr Peter Oxholm MD ( Medical Department TA, Division of Medical Rheumatology and Immunology, RHIMA Centre 7562, Rigshospitalet, National University Hospital, Tagensvej 20, DK-2200 Copenhagen N, Denmark.


A new model for classifying the clinical disease manifestations of primary Sjögren's syndrome is introduced. Three ‘exocrine’ and four ‘nonexocrine’ subgroups of disease manifestations are defined. Accordingly, ‘surface exocrine disease' includes the diagnostic features from eyes, mouth, and the manifestations from the upper airways, skin and genital tract. Involvement of the excretory parenchyma of the lungs, hepatobiliary system, pancreas, gastrointestinal tract and kidneys is designated ‘internal organ exocrine disease’. We suggest ‘monoclonal B lymphocyte disease’ to be an exocrine disease manifestation because it originates mostly from the immunoinflammatory foci of the autoimmune exocrinopathy. The nonexocrine manifestations are subgrouped into ‘inflammatory vascular disease’, ‘noninflammatory vascular disease’, ‘mediator-induced disease’ and ‘autoimmune endocrine disease’.