Troy E. Daniels

Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group

Classification criteria for Sjögrens syndrome (SS) were developed and validated between 1989 and 1996 by the European Study Group on Classification Criteria for SS, and broadly accepted. These have been re-examined by consensus group members, who have introduced some modifications, more clearly defined the rules for classifying patients with primary or secondary SS, and provided more precise exclusion criteria.

American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort.

We propose new classification criteria for Sjögrens syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS.

The histopathology of Sjögren's syndrome in labial salivary gland biopsies

Abstract Labial salivary gland biopsy specimens from seventy-five patients evaluated for Sjogrens syndrome were examined histologically. Clinically severe cases of Sjogrens syndrome showed the most marked histologic change. Focus score was found to be the most useful histologic index of severity of the disease. Large numbers of plasma cells were found in small foci and mild cases. Plasma cells were decreased in proportion, compared to lymphocytes, in larger foci and severe cases. These observations are discussed in the light of possible mechanisms of tissue damage in this disease.

The oral component of Sjögren's syndrome

This study reports the results of an interdisciplinary approach to the diagnosis of Sjögrens syndrome in 100 patients. Ocular and systemic diagnoses and oral features from the history and physical examination are correlated with measurements of stimulated parotid flow rate and labial salivary gland histopathology. A new diagnostic criterion is introduced whereby labial salivary gland focus scores are used to establish the presence of the oral component of Sjögrens syndrome in place of the subjective evaluation of xerostomia. The differential diagnosis of the oral clinical features of Sjögrens syndrome and the clinical management of the oral component of this disease are discussed.

A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's Syndrome International Registry.

PURPOSE To describe, apply, and test a new ocular grading system for assessing keratoconjunctivitis sicca (KCS) using lissamine green and fluorescein. DESIGN Prospective, observational, multicenter cohort study. METHODS The National Institutes of Health-funded Sjögrens Syndrome International Registry (called Sjögrens International Collaborative Clinical Alliance [SICCA]) is developing standardized classification criteria for Sjögren syndrome (SS) and is creating a biospecimen bank for future research. Eight SICCA ophthalmologists developed a new quantitative ocular grading system (SICCA ocular staining score [OSS]), and we analyzed OSS distribution among the SICCA cohort and its association with other phenotypic characteristics of SS. The SICCA cohort includes participants ranging from possibly early SS to advanced disease. Procedures include sequenced unanesthetized Schirmer test, tear break-up time, ocular surface staining, and external eye examination at the slit lamp. Using statistical analyses and proportional Venn diagrams, we examined interrelationships between abnormal OSS (>or=3) and other characteristics of SS (labial salivary gland [LSG] biopsy with focal lymphocytic sialadenitis and focus score >1 positive anti-SS A antibodies, anti-SS B antibodies, or both). RESULTS Among 1208 participants, we found strong associations between abnormal OSS, positive serologic results, and positive LSG focus scores (P < .0001). Analysis of the overlapping relationships of these 3 measures defined a large group of participants who had KCS without other components of SS, representing a clinical entity distinct from the KCS associated with SS. CONCLUSIONS This new method for assessing KCS will become the means for diagnosing the ocular component of SS in future classification criteria. We find 2 forms of KCS whose causes may differ.

Beta2 microglobulin and lymphocytic infiltration in Sjögren's syndrome.

We measured salivary beta2 microglobulin concentrations in 49 patients evaluated for Sjögrens syndrome with a labial salivary-gland biopsy. The salivary concentration was elevated and a significant correlation (P less than 0.001) was found between the concentration and the degree of inflammation seen in the biopsy. Serum concentrations were increased in 135 patients with Sjögrens syndrome. Striking elevations were seen in patients with associated renal or lymphoproliferative complications. Three patients have an increase in serum beta2 microglobulin concentration in association with exacerbation of their disease, and six a decrease after clinically efficacious therapy. These data indicate that determination of beta2 microglobulin in saiva may provide a simple, noninvasive technic for estimation of the degree of local inflammation in autoimmune disease.

Effectiveness of pilocarpine in postradiation xerostomia

Pilocarpine and placebo tablets were administered for 90 day periods in a double‐blind, sequential crossover trial to 12 patients with postradiation xerostomia. Salivary flow was measured by two techniques, symptomatic change and adverse side effects were also recorded. Nine of the 12 patients showed marked improvement by two or more criteria while taking pilocarpine. None of the 12 patients showed meaningful improvement while on placebo. Side effects were minimal and easily controlled. These results show that pilocarpine is effective in relieving the signs and symptoms of postradiation xerostomia. Cancer 59:1123‐1125, 1987.

Choristomas of the oral cavity: A review

The choristoma is a tumorlike mass of normal cells in an abnormal location. Intraoral choristomas have been reported under a wide variety of names. This comprehensive review of the English-language literature on oral choristomas offers a classification of these lesions, analyzes their clinical and histologic features, and discusses possible pathogeneses and treatment.

T and B Lymphocytes in Peripheral Blood and Tissue Lesions in Sjögren's Syndrome

Lymphocyte heterogeneity was studied in peripheral blood and salivary gland lesions in 24 patients with Sjögrens syndrome. Peripheral blood B cells, measured by immunofluorescence with specific antiserum to immunoglobulins or by rosette assay with complementcoated erythrocytes, were increased in most patients. Peripheral blood T cells, measured by immunofluorescence with rabbit antiserum to human thymocytes or by rosette assay with sheep erythrocytes, were reduced in eight patients. Three had associated rheumatoid arthritis, two had a generalized lymphoproliferative disorder, and one each had scleroderma, systemic lupus erythematosus, and neuropathy. The salivary gland lymphocytic infiltrates present in labial biopsy specimens were compared in 10 patients using an indirect immunofluorescent method with anti-human T cell serum and a quantitative focus-scoring method. In general, there was a correlation between the number of T cells and the extent of the infiltrate. Striking accumulations of T cells were present in some patients, but clusters of presumed B cells were also seen. These results indicate an increase in peripheral blood B cells in most patients, a decrease in T cells in some, and a mixed T and B cell infiltrate in the salivary gland lesions.

Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjogren's syndrome among 1,726 registry participants.

OBJECTIVE To examine associations between labial salivary gland (LSG) histopathology and other phenotypic features of Sjögrens syndrome (SS). METHODS The database of the Sjögrens International Collaborative Clinical Alliance (SICCA), a registry of patients with symptoms of possible SS as well as those with obvious disease, was used for the present study. LSG biopsy specimens from SICCA participants were subjected to protocol-directed histopathologic assessments. Among the 1,726 LSG specimens exhibiting any pattern of sialadenitis, we compared biopsy diagnoses against concurrent salivary, ocular, and serologic features. RESULTS LSG specimens included 61% with focal lymphocytic sialadenitis (FLS; 69% of which had focus scores of ≥1 per 4 mm²) and 37% with nonspecific or sclerosing chronic sialadenitis (NS/SCS). Focus scores of ≥1 were strongly associated with serum anti-SSA/SSB positivity, rheumatoid factor, and the ocular component of SS, but not with symptoms of dry mouth or dry eyes. Those with positive anti-SSA/SSB were 9 times (95% confidence interval [95% CI] 7.4-11.9) more likely to have a focus score of ≥1 than were those without anti-SSA/SSB, and those with an unstimulated whole salivary flow rate of <0.1 ml/minute were 2 times (95% CI 1.7-2.8) more likely to have a focus score of ≥1 than were those with a higher flow rate, after controlling for other phenotypic features of SS. CONCLUSION Distinguishing FLS from NS/SCS is essential in assessing LSG biopsies, before determining focus score. A diagnosis of FLS with a focus score of ≥1 per 4 mm², as compared to FLS with a focus score of <1 or NS/SCS, is strongly associated with the ocular and serologic components of SS and reflects SS autoimmunity.