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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).


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Web:

Sjögren's Syndrome Foundation

British Sjögren's Syndrome Association

Mayoclinic

NIAMS

Medscape

Swiss Medical Forum

MedicineNet

Arthritis and Sjögren - WebMD