Recognizing and Managing the Oral Clues That Point to Sjögren's Syndrome

, State University of New York at Buffalo

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In This Article

Diagnosis: Three Criteria Sets Compared

Currently, there are no universally accepted criteria for the diagnosis of SS. Published diagnostic guidelines form a wide spectrum and vary in their degree of astringency. The most restrictive are the San Diego Criteria, which require evidence of an autoimmune process associated with destruction of salivary and lacrimal gland tissues in order to render a diagnosis of SS. In contrast, the European Community Study Group and Copenhagen Criteria are the least stringent in that they are based on clinical findings of dry mouth and eyes but do not require gland biopsy or assessment of the patient for autoantibodies. Table IV compares the Copenhagen, San Diego, and European Community Criteria for the diagnosis of SS. Table V elaborates on the clinical and laboratory procedures used for the diagnosis of SS.

The European Community Criteria require 4 of the following 6 items to be present for SS to be diagnosed:

  • at least 1 specified ocular symptom;

  • at least 1 oral symptom;

  • abnormal findings on Schirmer's test or rose bengal stain;

  • abnormal results on salivary gland biopsy;

  • at least 1 abnormal result for unstimulated salivary flow rate, sialography, or scintigraphy; and

  • presence of at least 1 of the following antibodies, rheumatoid factor, ANA, SS-A or SS-B.[37]

According to the Copenhagen Criteria, a patient could be diagnosed with SS when 2 tests for xerophthalmia and 2 for xerostomia yield abnormal findings. No serologic and/or histopathologic evidence is required and no distinction is made between primary and secondary SS. At the other extreme of the spectrum, the more restrictive San Diego Criteria require that in addition to 2 abnormal ocular-test results, in order to make a diagnosis of primary SS there must be documentation of salivary gland hypofunction and serologic evidence of autoimmune disease. A "definite" diagnosis of SS is rendered only when a labial biopsy demonstrates abnormal findings. A provision of "possible" SS is conferred to cases in which no minor salivary gland biopsy is performed. A diagnosis of secondary SS is given when in addition to the lacrimal and salivary gland dysfunction, a connective tissue disease is documented.[38]

It is important to recognize that the Copenhagen Criteria may categorize a patient as having SS when in reality the salivary and lacrimal dysfunction may have a pharmacologic genesis and may not be related to an autoimmune process. In contrast, in the absence of serologic markers indicative of systemic autoimmunity, the San Diego Criteria exclude as having SS individuals with apparent lacrimal and salivary gland hypofunction. The European Community Criteria will identify more subjects as having SS, compared with the Copenhagen and San Diego Criteria.[37] Fox and Saito[38] have stressed that, because in the US a diagnosis of SS may have an impact on a patient's ability to obtain insurance and medical benefits, the diagnosis of SS should be reserved only for patients for whom there is irrefutable serologic evidence of SS. Furthermore, in the absence of an autoimmune disorder, the specter of lymphoma is weakened and these patients are spared unnecessary distress.

Serology. Laboratory tests of individuals with SS reflect a B-cell hyperactivity. Rheumatoid factor is present in the serum of most patients with primary or secondary SS, often in high titers. Antinuclear antibodies (ANA), detected by indirect immunofluorescence, are also often present in the serum of patients with SS, usually in a speckled or homogeneous pattern.[39] Antibodies against nuclear antigens Ro (SS-A) and La (SS-B) are found in many patients by the gel precipitation method, but they are identified in nearly all patients if sensitive, solid-phase, immunoadsorbent assays are used.[39,40]

Histopathology. Biopsy of minor salivary glands was introduced in 1966 as a clinical procedure for diagnosis of the salivary component of SS. The characteristic histopathologic features of minor salivary glands in SS are focal lymphocytic sialoadenitis (Figs. 6, 7). Aggregates of 50 or more lymphocytes (a focus) are seen adjacent to normal-appearing acini. Using a semiquantitative inflammatory grading method, Chisholm and Mason[41] graded inflammation in labial salivary gland biopsy specimens and found that grade IV inflammation (more than 1 focus /4mm2 area of gland) was seen only in patients with SS. This histopathologic observation (focus score greater than 1 focus/4mm2) in a labial salivary gland specimen is regarded as the most disease-specific diagnostic criterion for the salivary component of SS.

Figure 6. Histopathology (H&E, original magnification x 100). Low magnification of minor salivary gland lobules showing extensive lymphocytic infiltration (Grade IV, according to Chisholm and Mason's criteria).
Figure 7. Histopathology (H&E, original magnification x 200). The chronic inflammatory cell infiltrate is comprised of lymphocytes, plasma cells, and macrophages. Two excretory ducts and 3 mucous salivary gland acini are seen (Grade IV, according to Chisholm and Mason's criteria).

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