Sjogren's Syndrome info

DIAGNOSIS OF SJOGREN'S SYNDROME 

 

Sjogren's Syndrome is considered the second most common autoimmune rheumatic disorder.

Many people with auto immune diseases find diagnosis is often delayed for years as the symptoms are misinterpreted or overlooked.

The problem with diagnosis is that it is much easier when the syndrome is more advanced rather than in the early stage. Also if the main presenting symptoms include muscle pain or head aches then the dryness, which is important in doctor's minds, can be overlooked.

 

The early stages of Sjogren's Syndrome are often picked up by an opthalmologist (dry eyes, blepharitis, adies tonic) or a dentist (dry mouth, many cavities).

Often, a rheumatologist (a doctor who specialises in diseases of the joints, muscles, and bones) may diagnose the syndrome.

 

Tests for Sjogren's Syndrome

No single test is specific in diagnosing SS.

Schirmer's Test - checks the dryness of the eyes with a simple test using thin paper.

Blood tests that may be required.

ANA test - Antinuclear Antibody test - 40% to 70% of patients with SS have a positive ANA test result. This result supports the diagnosis but is not required for diagnosis. The doctor may want to test for SSA and SSB subsets of ANA. "The frequency of autoantibodies to SSA in patients with Sjögren’s can be 90% or greater if the test is done by enzyme immunoassay. " Lab Tests Online

Autoantibodies are antibodies created by the immune system that attack the body rather than a foreign object.

More info on diagnosis:

How To Diagnose And Manage Sjogren's Syndrome (they read it out loud to you) By Lynn M. Petruzzi and Frederick B. Vivino

Classification and diagnosis of Sjögren's syndrome-I by Robert Fox, MD, PhD and Paul Creamer, MD

2016 ACR-EULAR Classification Criteria for primary Sjögren’s Syndrome

I. Ocular Symptoms (at least one)

  • Symptoms of dry eyes for at least 3 months
  • A foreign body sensation in the eyes
  • Use of artificial tears 3 or more times per day

II. Oral Symptoms (at least one)

  • Symptoms of dry mouth for at least 3 months
  • Recurrent or persistently swollen salivary glands
  • Need for liquids to swallow dry foods

III. Ocular Signs (at least one)

  • Abnormal Schirmer’s test, (without anesthesia; ≤5 mm/5 minutes)
  • Positive vital dye staining of the eye surface

IV. Histopathology

  • Lip biopsy showing focal lymphocytic sialoadenitis (focus score ≥1 per 4 mm2)

V. Oral Signs (at least one)

  • Unstimulated whole salivary flow (≤1.5 mL in 15 minutes)
  • Abnormal parotid sialography
  • Abnormal salivary scintigraphy

VI. Autoantibodies (at least one)

  • Anti-SSA (Ro) or Anti-SSB (La), or both
These criteria were not developed for use in clinical practice and not every patient who receives a clinical diagnosis of SS will fulfill these criteria. They were developed for clinical trials.  

The American-European consensus proposed rules for classification of patients based on the criteria are as follows:

  •  Primary Sjögren's syndrome:

For patients with no associated connective tissue or autoimmune disease (eg, RA, SLE) and no exclusionary diagnoses (see "Exclusion Criteria" below) a classification of primary SS is made according to the consensus rules in one of the three following ways:

1. The patient has either a positive salivary gland biopsy result or autoantibodies, and satisfies a total of 4 of the six items. 

2. Alternatively, the patient satisfies three of the four objective items (ocular signs, biopsy, salivary gland involvement, or autoantibodies). 

3. Finally, a classification tree with sensitivity of 96 percent and specificity of 94 percent can be used to determine whether or not patients are classified as having primary SS.

  •  Secondary Sjögren's syndrome:

A classification of secondary SS is made according to the consensus rules if a "well defined" connective tissue disease is present and at least one symptom item (indicative of ocular or oral dryness) and any two of the three objective items exclusive of autoantibodies (ie, ocular signs, biopsy, or tests of salivary gland involvement other than biopsy) are present.


Exclusion Criteria

  • Past head and neck radiation treatment
  • Hepatitis C infection
  • Acquired immunodeficiency syndrome (AIDS)
  • Pre-existing lymphoma
  • Sarcoidosis
  • Graft versus host disease
  • Current use of anticholinergic drugs
More Info on criteria:

Classification criteria in Sjögren’s syndrome

2016 ACR-EULAR Classification Criteria for primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts

The 2016 classification criteria for primary Sjogren’s syndrome: what’s new?

 

 

There are 2 kinds of Sjogren's Syndrome - Primary and Secondary