British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease.

Sjögren disease (SD) is a chronic, autoimmune disease of unknown aetiology with significant impact on quality of life. Although dryness (sicca) of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations are common. The key management aim should be to empower the individual to manage their condition-conserving, replacing and stimulating secretions; and preventing damage and suppressing systemic disease activity. This guideline builds on and widens the recommendations developed for the first guideline published in 2017. We have included advice on the management of children and adolescents where appropriate to provide a comprehensive guideline for UK-based rheumatology teams.

The management of Sjögren's syndrome: British Society for Rheumatology guideline scope.

The guideline will be developed using the methods and processes outlined in Creating Clinical Guidelines: Our Protocol [1]. This development process to produce guidance, advice and recommendations for practice has National Institute for Health and Care Excellence (NICE) accreditation.

The development and validation of the King's Sarcoidosis Questionnaire for the assessment of health status.

RATIONALE: Health status is impaired in patients with sarcoidosis. There is a paucity of tools that assess health status in sarcoidosis. The objective of this study was to develop and validate the King's Sarcoidosis Questionnaire (KSQ), a new modular health status measure. METHODS: Patients with sarcoidosis were recruited from outpatient clinics. The development of the questionnaire consisted of three phases: item generation; item reduction, Rasch analysis to create unidimensional scales and validation; repeatability testing. RESULTS: 207 patients with sarcoidosis (organ involvement: 184 lung, 54 skin, 45 eye disease) completed a 65-item preliminary questionnaire. 36 items were removed due to redundancy or poor fit to the Rasch model. The final version of the KSQ consisted of five modules (General health status, Lung, Skin, Eye, Medications). Internal consistency assessed with Cronbach's α coefficient was 0.70-0.93 for KSQ modules. Concurrent validity of the Lung module was high compared with St George's Respiratory Questionnaire (r=-0.83) and moderate when compared to forced vital capacity (r=0.49). Concurrent validity with skin-specific and eye-specific measures ranged from r=-0.4 to 0.8. The KSQ was repeatable over 2 weeks (n=39), intraclass correlation coefficients for modules were 0.90-0.96. CONCLUSIONS: The KSQ is a brief, valid, self-completed health status measure for sarcoidosis. It can be used in the clinic to assess sarcoidosis from the patients' perspective.

Clinical safety of a minor eye conditions scheme in England delivered by community optometrists.

OBJECTIVE: The aim of this study was to monitor the activity and evaluate the clinical safety of a minor eye conditions scheme (MECS) conducted by accredited community optometrists in Lambeth and Lewisham, London. METHODS AND ANALYSIS: Optometrists underwent an accredited training programme, including attendance at hospital eye services (HES) clinics. Patients who satisfied certain inclusion criteria were referred to accredited MECS optometrists by their general practitioners (GPs) or could self-refer. Data were extracted from clinical records. A sample of MECS clinical records was graded to assess the quality of the MECS optometrists' clinical management decisions. Referrals to the HES were assessed by the collaborating ophthalmologists and feedback was provided. RESULTS: A total of 2123 patients (mean age 47 years) were seen over 12 months. Two-thirds of the patients (67.3%) were referred by their GP. The most common reasons for patients needing a MECS assessment were 'red eye' (36.7% of patients), 'painful white eye' (11.1%), 'flashes and floaters' (10.2%); 8.7% of patients had a follow-up appointment. Of the patients seen, 75.1% were retained in the community, 5.7% were referred to their GP and 18.9% were referred to the HES. Of the HES referrals, 49.1% were routine, 22.6% urgent and 28.3% emergency. Of the records reviewed, 94.5% were rated as appropriately managed; 89.2% of the HES referrals were considered appropriate. CONCLUSION: The findings of this study indicate that optometrists are in a good position to work very safely within the remits of the scheme and to assess risk.

Association of Dry Eye Tests With Extraocular Signs Among 3514 Participants in the Sjögren's Syndrome International Registry.

PURPOSE: To identify a screening strategy for dry eye patients with a high likelihood of having Sjogren syndrome (SS) through the evaluation of the association of ocular surface tests with the extraocular signs used for the diagnosis of SS. DESIGN: Multicenter cross-sectional study. METHODS: The Sjogren's International Clinical Collaborative Alliance (SICCA) registry enrolled 3514 participants with SS or possible SS from 9 international academic sites. Ocular surface evaluation included Schirmer I testing, tear breakup time (TBUT), and staining of the cornea (0-6 points) and conjunctiva (0-6 points). Multivariate logistic regression analysis was performed to identify predictive factors for (1) histopathologic changes on labial salivary gland (LSG) biopsies (positive = focus score of ≥1 focus/4 mm2) and (2) positive anti-SSA/B serology. RESULTS: The adjusted odds of having a positive LSG biopsy were significantly higher among those with an abnormal Schirmer I test (adjusted OR = 1.26, 95% CI 1.05-1.51, P = .014) and positive conjunctival staining (for each additional unit of staining 1.46; 95% CI 1.39-1.53, P < .001) or corneal staining (for each additional unit of staining 1.14; 95% CI 1.08-1.21, P < .001). The odds of having a positive serology were significantly higher among those with an abnormal Schirmer I test (adjusted OR = 1.3; 95% CI 1.09-1.54, P = .004) and conjunctival staining (adjusted OR = 1.51; 95% CI 1.43-1.58, P < .001). CONCLUSIONS: In addition to corneal staining, which was associated with a higher likelihood of having a positive LSG biopsy, conjunctival staining and abnormal Schirmer I testing are of critical importance to include when screening dry eye patients for possible SS, as they were associated with a higher likelihood of having a positive LSG biopsy and serology.

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.

Bilateral endogenous endophthalmitis after holmium laser lithotripsy.

Endogenous endophthalmitis is a potentially blinding condition that occurs after the spread of organisms to the eye from a focus of infection elsewhere in the body. The holmium laser has gained increasing acceptance as being safe and effective for endoscopic lithotripsy. We report what we believe to be the first time endogenous endophthalmitis has been described as occurring after holmium laser lithotripsy, although it has been reported after extracorporeal shock wave lithotripsy. This 55-year-old woman developed infections in both eyes 2 to 3 weeks after the lithotripsy, with a good response to appropriate antibiotic treatment.

Asymptomatic choroidal granulomas in common variable immunodeficiency.

Common variable immunodeficiency disease represents the most common cause of significant antibody deficiency in adults. It is a heterogeneous group of disorders that can be sporadic or familial with various modes of inheritance with defects in B-cell function, regulatory T-cells and macrophage function all being reported. Herein a case of asymptomatic bilateral choroidal granulomatous disease in a 27-year-old man with common variable immunodeficiency disease is described.

Pseudo-hypopyon as the presenting feature in B-cell and T-cell intraocular lymphoma.

The clinicopathologic correlation of two patients with primary intraocular lymphoma is described, both of whom had a rare and unusual presentation of hypopyon uveitis. Sampling of aqueous fluid proved the cytopathologic diagnosis of B-cell lymphoma in the first patient. In the second patient an iris biopsy confirmed a T-cell lymphoma. The samples were examined using haematoxylin and eosin, and immunohistochemical stains. These patients were diagnosed with primary intraocular lymphoma on initial presentation with hypopyon uveitis. Secondary glaucoma was noted in the patient with T-cell lymphoma. Both cases appeared confined to the eye and initially responded favourably to aggressive chemotherapy and radiotherapy, but later went on to develop systemic involvement. Clinicians should be aware of this dramatic mode of presentation, which is unusual for ocular lymphoma.