4 Interesting Facts of Sjogrens Syndrome
1. Sjogrens Syndrome (SS) is a common autoimmune disease in women and should be considered in any patient with unexplained symptoms and a positive antinuclear antibody (ANA).
2. The most common symptoms are keratoconjunctivitis sicca (KCS), xerostomia, and parotid gland swelling; however, extraglandular features including lymphoma do occur.
3. Antibodies against Ro/SS-A and La/SS-B are the serologic hallmarks of primary SS.
4. A minor salivary gland lip biopsy showing a chronic lymphocytic infiltrate is the diagnostic gold standard.
Who was Sjogren and what is his syndrome?
Henrich Sjögren was born in 1899 in Stockholm and received his M.D. from the Karolinska Institute in 1927. His description of keratoconjunctivitis sicca (KCS) and arthritis serves as the basis for SS. SS has also been known as Mikulicz’s disease (which is now considered by some experts to represent early descriptions of IgG4-related disease), Gougerot’s syndrome, sicca syndrome, and autoimmune exocrinopathy. SS refers to a slowly progressive, autoimmune disease that primarily affects exocrine organs (lacrimal, salivary, and parotid glands). Lymphocytes infiltrate these organs, resulting in decreased secretions from glandular destruction and effects on neural stimulation.