ICD-11

Nonorgan specific systemic autoimmune diseases which do not fulfil the diagnostic criteria for any single recognised disease entity.

Sjögren syndrome (SS) is a slowly progressive, systemic inflammatory autoimmune disease affecting primarily the exocrine glands. Lymphocytic infiltrates replace functional epithelium, leading to oral and ocular dryness. Characteristic autoantibodies (e.g., anti-Ro/SS-A and/or anti-La/SS-B) are produced. The disorder can occur alone (it is then known as “primary SS”) or in association with another autoimmune disease (it is then known as “secondary SS”).

Primary Sjögren syndrome is a progressive, systemic inflammatory autoimmune disease affecting primarily the exocrine glands in the absence of other systemic autoimmune diseases. Extraglandular manifestations, which can be divided into two categories: periepithelial organ involvement (i.e., interstitial nephritis, liver involvement), and obstructive bronchitis, is the result of lymphocytic invasion in the epithelia of organs beyond the exocrine glands. Extraepithelial manifestations, such as palpable purpura, glomerulonephritis, and peripheral neuropathy, are produced by an immune complex deposition resulting from the ongoing B-cell hyper-reactivity. The latter manifestations are associated with increased risk for developing lymphoma.