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Primary Sjögren's syndrome: the challenge for classification of disease manifestations
Author(s) -
OXHOLM P.,
ASMUSSEN K.
Publication year - 1996
Publication title -
journal of internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.625
H-Index - 160
eISSN - 1365-2796
pISSN - 0954-6820
DOI - 10.1046/j.1365-2796.1996.482818000.x
Subject(s) - medicine , exocrine gland , disease , autoimmune disease , pathology , systemic disease , gastrointestinal tract , vascular disease , immunopathology , igg4 related disease , endocrine system , pancreas , immunology , hormone , secretion
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’.