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.
Primary Sjögren's syndrome: the challenge for classification of disease manifestations
Article first published online: 31 OCT 2003
Blackwell Science Ltd
Journal of Internal Medicine
Volume 239, Issue 6, pages 467–474, June 1996
How to Cite
OXHOLM, P. and ASMUSSEN, K. (1996), Primary Sjögren's syndrome: the challenge for classification of disease manifestations . Journal of Internal Medicine, 239: 467–474. doi: 10.1046/j.1365-2796.1996.482818000.x
- Issue published online: 31 OCT 2003
- Article first published online: 31 OCT 2003
- Cited By
- classification criteria;
- disease manifestations;
- Sjögren's syndrome
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’.