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

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'.