[New international consensus statement about the definition, classification, ethiology, diagnostics and therapy of dry eye (TFOS DEWS II)]. / Új nemzetközi konszenzusnyilatkozat a száraz szem definíciójáról, felosztásáról, etiológiájáról, diagnosztikájáról és terápiájáról.

Ten years have passed since the publication of the DEWS Report that summarized the information based on scientific literature concerning dry eye disease. Hundreds of papers have been published since then and time has come for a new summary. Organized by the Tear Film & Ocular Surface Society, 12 working groups summerized former and recent data. The DEWS II Report was created. The authors of the present publication summarize the most important changes in definition, classification, diagnostics, and therapy concerning dry eye disease. They also disclose the relevant changes on which the non-ophthalmologist specialists have to be informed. The DEWS II Report published by TFOS consists of 11 chapters. Completely new chapters deal with the role of sensation/pain and iatrogenic dry eyes. Orv Hetil. 2018; 159(20): 775-785.

Automated Grading System for Evaluation of Superficial Punctate Keratitis Associated With Dry Eye.

PURPOSE: To develop an automated method of grading fluorescein staining that accurately reproduces the clinical grading system currently in use. METHODS: From the slit lamp photograph of the fluorescein-stained cornea, the region of interest was selected and punctate dot number calculated using software developed with the OpenCV computer vision library. Images (n = 229) were then divided into six incremental severity categories based on computed scores. The final selection of 54 photographs represented the full range of scores: nine images from each of six categories. These were then evaluated by three investigators using a clinical 0 to 4 corneal staining scale. Pearson correlations were calculated to compare investigator scores, and mean investigator and automated scores. Lin's Concordance Correlation Coefficients (CCC) and Bland-Altman plots were used to assess agreement between methods and between investigators. RESULTS: Pearson's correlation between investigators was 0.914; mean CCC between investigators was 0.882. Bland-Altman analysis indicated that scores assessed by investigator 3 were significantly higher than those of investigators 1 and 2 (paired t-test). The predicted grade was calculated to be: Gpred = 1.48log(Ndots) - 0.206. The two-point Pearson's correlation coefficient between the methods was 0.927 (P < 0.0001). The CCC between predicted automated score Gpred and mean investigator score was 0.929, 95% confidence interval (0.884-0.957). Bland-Altman analysis did not indicate bias. The difference in SD between clinical and automated methods was 0.398. CONCLUSIONS: An objective, automated analysis of corneal staining provides a quality assurance tool to be used to substantiate clinical grading of key corneal staining endpoints in multicentered clinical trials of dry eye.

Do we need new diagnostic criteria for Sjögren's syndrome?

Diagnostic and classification criteria for Sjögren's syndrome (SS) continue to evolve as more is learned about SS and about autoimmune diseases in general. Among diagnostic or classification criteria for SS that are in current use, most include various and variable combinations of results from questions about symptoms and objective tests, many of which are not specific to SS. Given the rapid increase of genetic knowledge about other autoimmune diseases and the potential of finding and testing new biological agents to treat SS, selection of patients who have as uniform a disease process as possible becomes an important goal to better understand and treat this prevalent autoimmune disease. Such is the goal and promise of the latest entry into the SS classification criteria field.

[Epidemiology of primary Sjörgren's syndrome]. / Epidemiologie des primären Sjögren-Syndroms.

According to the classification criteria of the American-European Consensus Group (AECG), the prevalence of primary Sjögren's syndrome (pSS) of about 0.2% in the adult population and a yearly incidence of 4/100.000 in the general population are far lower than previously assumed. Moreover, the repeatedly reported male/female ratio of 1:9 seems to lie more in the range of 1:20. Male pSS patients show fewer immunological, histopathological or sialographic findings and organ involvement. Information on age at disease onset has also changed over the last decade. Recent studies indicate an onset age of approximately 45 years as compared to 56 in earlier studies of the last decade. Patients with an early disease onset are more frequently positive for rheumatoid factor (RF) and/or anti-Ro/SS-A. These patients also seem to have a higher risk of developing hypocomplementemia or lymphadenopathy. As compared to earlier cohorts, the introduction of the rather specific AECG criteria will probably result in the participation of fewer men, younger patients in general and of more seriously ill patients in future cohorts. The change in the spectrum of pSS patients obviously reflects the altered classification criteria since the AECG criteria require anti-Ro/La positivity and therefore exclude a high number of patients with other immunological markers who also show severe sicca symptoms and organ involvements. About 5%-10% of pSS patients in rheumatological care suffer from severe extraglandular manifestations, which generally occur soon after disease onset. In particular, palpable purpura, hypocomplementemia, cryoglobulinemia and lymphoma are associated with increased mortality. In Germany, approximately one tenth of Sjögren syndrome patients receive specialized rheumatological care. There is still insufficient knowledge about the vast majority of pSS patients who are not treated by rheumatologists. These patients, as well as all those who, according to the AECG criteria, are not classified as having pSS either due to anti-Ro/La negativity or having secondary Sjögren's syndrome, probably add up to at least 0.4% of the adult population which, at present, suffers from considerable immunopathologic sicca symptoms.

[Is bioptic assurance reasonable in patients with Sjögren's syndrome? From focus score to diagnosing vasculitides]. / Ist eine bioptische Sicherung beim Sjögren-Syndrom sinnvoll? Vom Focus-Score zur Vaskulitisdiagnostik.

Sjögren's syndrome is an autoimmune disease which targets the salivary and lacrimal glands in particular, causing sicca syndrome. Extraglandular manifestations are often seen. Chronic sialadenitis of the parotid gland is the most common symptom to be assessed for differential diagnosis. Common HE and Giemsa slices are histopathologically examined and graduated for lymphocyte infiltration (focus): grade 0: absent, grade 1: slight, grade 2: moderate non-focal infiltration, grade 3: 1 focus (> or =50 lymphocytes) per 4 mm2, grade 4: >1 focus. Grade 3 infiltrates correspond to a focus score of 1, which is one of four disease-classifying criteria acknowledged for diagnosis. Bioptic examination is also performed to rule out different (non-) immunologic sialadenitises, such as the necrotizing or epithelioid-like form (in sarcoidosis), and the extranodal marginal-zone lymphoma. Extraglandular manifestations of Sjögren's syndrome can also be safely diagnosed by histopathological examination. Emphases lie on vasculitides and myositides. Bioptic work-up, therefore, is not only reasonable but also an essential tool for diagnostics in Sjögren's syndrome.

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 Sjögren's Syndrome International Registry (called Sjögren's 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.

Classification and diagnosis of dry eye.

BACKGROUND: Dry eye, or keratoconjunctivitis sicca (KCS), is divided into two subgroups, tear-deficient and evaporative. Each form calls for a different therapeutic approach and it is therefore essential to apply a combination of diagnostic tests in order to establish the exact diagnosis. MATERIAL AND METHODS: The diagnosis of KCS is based in part on the patient's history and symptoms and in part on the application of specific tests. Several non-invasive tests exist (e.g. slit-lamp examination, meniscometry, interferometry). Mildly invasive tests are the fluorescein tests, staining with lissamine green, meibometry and meibography. Markedly invasive tests include the Schirmer test and staining with rose bengal. Additional histological procedures are the ocular ferning test and impression cytology. RESULTS: A combination of diagnostic tests leads to one of the two forms of KCS. Its severity is calculated according to grading systems, which exist for several tests. The longitudinal observation of the dry eye patient is provided on the basis of this same grading system, although limited reproducibility is reported for some tests. CONCLUSION: The diagnostic steps for dry eye patients can be efficiently arranged. In most of the cases, non-invasive or mildly invasive tests provide an accurate diagnosis.

Primary Sjögren's syndrome in children and adolescents: are proposed diagnostic criteria applicable?

OBJECTIVE: To compare the proposed criteria for the diagnosis of primary Sjögren's syndrome (pSS) in childhood to the validated American-European Consensus Group (AECG) classification criteria for pSS in adults. METHODS: Charts of 7 children with pSS seen at British Columbia's Children's Hospital (BCCH) and data on 128 children identified through Medline in the English language literature between 1963 and 2003 were reviewed for pediatric and AECG criteria for pSS. The presence of > or = 4 criteria was required to satisfy the respective classification criteria. The expert clinical opinion of pediatric rheumatologists was considered the gold standard for diagnosis. RESULTS: A total of 24/62 (39%) cases satisfied the AECG criteria; 47/62 (76%) satisfied the proposed pediatric criteria. Inclusion of recurrent parotitis increased the sensitivity of the pediatric clinical criteria. From the cases, 78/133 (59%) satisfied the pediatric oral symptom criteria; only 6/78 (8%) had xerostomia in the absence of recurrent parotitis. There was no reported case of recurrent conjunctivitis in the absence of keratoconjunctivitis sicca. We found 101/130 (78%) cases had at least one positive autoantibody test result [antinuclear antibodies (ANA), rheumatoid factor (RF), SSA, SSB]; 78/123 (63%) had autoantibodies to SSA or SSB. CONCLUSION: The AECG adult criteria for pSS should not be applied to children as the sensitivity is unacceptably low. The inclusion of recurrent parotitis increases the sensitivity of the pediatric criteria, and recurrent parotitis should alert the clinician to the possibility of pSS. The inclusion of recurrent conjunctivitis did not improve the sensitivity over the AECG ocular criteria. The addition of ANA and RF to the AECG criteria did not change the number of patients satisfying the criteria for pediatric pSS.