PRIMARY SJOGRENS-SYNDROME - THE CHALLENGE FOR CLASSIFICATION OF DISEASE MANIFESTATIONS

A new model for classifying the clinical disease manifestations of pri mary Sjogren's syndrome is introduced. Three 'exocrine' and four 'none xocrine' 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 genit al tract. Involvement of the excretory parenchyma of the lungs, hepato biliary system, pancreas, gastro-intestinal tract and kidneys is desig nated 'internal organ exocrine disease'. We suggest 'monoclonal B lymp hocyte disease' to be an exocrine disease manifestation because it ori ginates mostly from the immunoinflammatory foci of the autoimmune exoc rinopathy. The nonexocrine manifestations are subgrouped into 'inflamm atory vascular disease', 'noninflammatory vascular disease', 'mediator -induced disease' and 'autoimmune endocrine disease'.