Wallach's Interpretation of Diagnostic Tests: Pathways to Arriving at a Clinical Diagnosis (17 page)

   ESR and CRP are elevated.

Suggested Reading

Aletaha D, Neogi T, Silman AJ, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative.
Arthritis Rheum.
2010;62(9):2569–2581.

SJÖGREN SYNDROME

   Definition

   Sjögren syndrome (SjS) is an inflammatory, autoimmune disease in which the exocrine glands, mainly the salivary and lacrimal glands, are attacked and destroyed by the immune cells.

   The syndrome is divided into primary SjS (not associated with other diseases), or secondary SjS, which is associated with other autoimmune rheumatic conditions, principally rheumatoid arthritis (most common) or SLE. In both primary and secondary SjS, decreased exocrine gland function leads to the “sicca complex,” characterized by dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca).

   The American-European Consensus Group criteria for classification of SjS were developed in 2002. In 2012, new criteria were proposed by the American College of Rheumatology and the Sjögren’s International Collaborative Clinical Alliance. Using the later criteria, classification of SjS, which applies to individuals with signs/symptoms that may be suggestive of SjS, will be met in the presence of at least two of the following three objective features:

   Positive serum anti-SSA/Ro and/or anti-SSB/La
or
positive rheumatoid factor and ANA titer ≥1:320

   Labial salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score ≥1 focus/4 mm
²

   Keratoconjunctivitis sicca 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 last 5 years)

   Who Should Be Suspected?

   SjS patients typically present with complaints of ocular symptoms, such as persistent dry eyes for more than 3 months and oral symptoms of dryness (e.g., the need to drink water to be able to swallow food). This may be associated with vague symptoms such as fatigue and myalgia.

   SjS can affect people of any age, but symptoms usually appear between the ages of 45 and 55. It affects 10 times as many women as men, and about half of SjS patients also have rheumatoid arthritis or other connective tissue diseases, such as lupus.

   Occasionally, other tissues or organs may be involved. As a result, extraglandular manifestations develop, and they may include pain and stiffness in the joints, even in the absence of rheumatoid arthritis or lupus, rashes on the arms and legs related to vasculitis, or inflammation in the lungs, liver, and kidneys.