Sjögren’s Syndrome (SS)
Henrich Sjögren was born in 1899 in Stockholm. In 1933, he published a monograph associating dry eyes with arthritis. SS has also been known as Mikulicz’s disease, Gougerot’s syndrome, sicca syndrome, and autoimmune exocrinopathy.
Sjögren’s syndrome is the most common autoimmune disease and should be considered in any patient with unexplained symptoms and a positive antinuclear antibody
The most common symptoms are keratoconjunctivitis sicca, xerostomia, and parotid gland swelling but any organ can be involved.
Antibodies against Ro/SS-A and La/SS-B are the serologic hallmarks of primary Sjögren’s syndrome.
A minor salivary gland lip biopsy showing a chronic lymphocytic infiltrate is the diagnostic gold standard.
Manifestations can be divided into local sicca symptoms and systemic extraglandular manifestations. The initial clinical manifestations of primary SS are as follows:
Sicca symptoms: Xerophthalmia (47%), Xerostomia (42%), Parotid gland enlargement (24%) Dyspareunia (5%)
Systemic manifestations: Arthralgias/arthritis (28%) Raynaud’s phenomenon (21%)
Fever/fatigue (10%) Lung involvement (2%) Kidney involvement (1%)
American College of Rheumatology classification criteria for SS:
The classification of SS, which applies to individuals with signs/symptoms that may be suggestive of SS, will be met in patients who have at least two of the following three objective features:
Positive serum anti-SSA/R0o and/or anti-SSB/La or a positive rheumatoid factor and ANA titer ≥1:320.
Labial salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score ≥1 focus/4 mm2.
Keratoconjunctivitis sicca (KCS) with ocular staining score ≥3 (assuming that the individual is not currently using daily eye drops for glaucoma and has not had corneal surgery or cosmetic eyelid surgery in the past 5 years).