Generally, the syndrome has an indolent or slowly
progressive course with the disease confined to the exocrine glands. In
30% of patients, general signs occur, which can involve neurological
function (abnormal gait, autonomic dysfunction, seizures, movement disorder,
ataxia/incoordination, insensitivity to pain, hyporeflexia, and even
paraparesis or quadriparesis), immunological disorder (immune system
anomalies, dysfunction autoimmunity), pharyngeal abnormality, respiratory
tract modification (interstitial-like disease), chronic atrophic gastritis,
celiac-like disease, distal renal tubular acidosis, Raynaud phenomenon
(35% of cases), and other skin anomalies. Lymphoma and mortality are
higher in these patients. All of these general signs are usually moderate.
Secondary Sjögren syndrome occurs in 10 to 20% of patients with
Rheumatoid Arthritis, Systemic Lupus Erythematosus, and Scleroderma. Various
other diseases can imitate this syndrome, including Sarcoidosis,
Lipoproteinemia, and Amyloidosis.