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 a 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.
Ultrasound and a minor salivary gland lip biopsy showing a chronic lymphocytic infiltrate is the diagnostic gold standard.
https://pubmed.ncbi.nlm.nih.go...
https://pubmed.ncbi.nlm.nih.go...
https://link.springer.com/chap...
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).
Web:
British Sjögren's Syndrome Association
Parotid Gland Ultrasound guided Biopsy
British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease