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.

Impact of Clinician Subjectivity on the Assessment of Dry Eye Disease Prevalence in a UK Public Health Care Patient Population.

Purpose: To understand the impact of subjectivity on diagnosis rates of dry eye disease (DED) in an unbiased population. Patients and Methods: A multicenter study enrolled 818 subjects with complete report forms (465 females, 67.1 ± 16.7 years, 353 males, 65.0 ± 15.9 years). Subjects were evaluated for staining, TBUT, tear osmolarity, meibomian gland disease, and OSDI. Results: Physicians diagnosed 48.7% of subjects as having DED, ranging from 42.9% to 62.3% between sites. Positivity rates for staining (≥ grade 1) ranged from 41.3% to 84.1% (mean = 0.8 ± 0.9 grade), TBUT (<10s) ranged from 39.1% to 61.6% (mean = 10.4 ± 6.6 seconds), osmolarity (>308 mOsm/L) ranged from 63.7% to 72.4% (mean = 319.7 ± 20.8), MGD grading ranged from 28.9% to 51.3% (mean = 0.5 ± 0.7), and symptoms measured by OSDI ranged from 57.6% to 71.0% (mean = 23.5 ± 20.5) between sites. Tear osmolarity was the most consistent between sites (max/min positivity = 114%), followed by OSDI (123%), TBUT (158%), MGD (178%), and staining (204%). DED markers were uncorrelated (average r2 = 0.05 ± 0.07). A substantial number of subjects (N = 110) exhibited positive symptoms (OSDI = 32.4 ± 15.7) and hyperosmolarity (338.1 ± 20.1 mOsm/L) but no other obvious signs of DED (MGD grade = 0.2 ± 0.4, TBUT = 13.5 ± 7.0 seconds, staining grade = 0.4 ± 0.5). Conclusion: Subjective signs of DED varied considerably, whereas objective measurements of OSDI and osmolarity were the most consistent between sites. A large proportion of subjects exhibited high symptoms and hyperosmolarity but no other obvious signs of dry eye disease, most of whom were undiagnosed by clinical assessment without access to the osmolarity measurement.

Hydroxychloroquine retinopathy screening guidelines: a false positive.

Hydroxychloroquine sulphate (HCQ) is widely used for the treatment of a variety of rheumatological and dermatological conditions. Despite the advantages of HCQ as a treatment option, it is important to be aware of its potential retinal toxicity, which may be irreversible and progressive. In December 2020, The Royal College of Ophthalmologists published revised recommendations on monitoring HCQ retinopathy. Our case report highlights some of the shortcomings of blindly following their monitoring algorithm by presenting a case where apparent HCQ retinopathy resolved after Yttrium Aluminium Garnet (YAG) laser capsulotomy. The case reiterates the importance of thorough clinical examination. We suggest that while the acquisition of the spectral domain optical coherence tomography and fundus autofluorescence may be objective, their interpretation is subjective. Even with the use of artificial intelligence algorithms, false positives may be generated if the tests are confounded by copathology. There is no gold-standard test for detecting HCQ toxicity.

Medial Pivot Designs Versus Conventional Bearing Types in Primary Total Knee Arthroplasty: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: Medial pivot (MP) designs are growing in popularity. They provide increased sagittal plane stability and theoretically replicate some aspects of native joint kinematics, which may improve total knee arthroplasty outcomes. METHODS: A systematic review was performed of randomized controlled trials (RCTs) that compared MP designs with cruciate-retaining, posterior-stabilized (PS), ultracongruent, or mobile-bearings in primary total knee arthroplasty, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The primary outcome measures were all clinical function scores, patient-reported outcome measures, and range of motion. The secondary outcome was complications. Two authors independently selected studies, performed data extraction, and risk-of-bias assessment. Studies at high risk of bias were excluded from meta-analysis. Treatment effects were assessed using random-effects meta-analysis and quantified using pooled mean differences or incidence rate differences as appropriate. RESULTS: Eight RCTs met inclusion criteria. Five compared MP with PS, two with ultracongruent, and one with cruciate-retaining and mobile-bearing. In total, 350 knees were randomized to MP and 375 to conventional bearings. One RCT was excluded from meta-analysis because of high risk of bias. Meta-analysis comparing MP with PS only was possible and found no differences at any time points for any outcome measure, including 2-year follow-up for Oxford Knee Score (MD = 0.35 favoring PS; 95% CI -0.49 to 1.20) and range of motion (MD = 1.58 favoring MP; 95% CI -0.76 to 11.92, P = 0.30) and 12 months for Western Ontario Arthritis Index (MD = 4.42 favoring MP; 95% CI -12.04 to 3.20, P = 0.09). CONCLUSIONS: There is no difference in clinical outcomes, with contemporary measurement tools, at any time points, between MP and PS. There are insufficient RCTs comparing MP with other bearings.

An objective method of diagnosing hydroxychloroquine maculopathy.

BACKGROUND: Hydroxychloroquine (HCQ) maculopathy is irreversible; primary prevention is done by regular monitoring. Guidelines of the Royal College of Ophthalmologists identify definite toxicity as having abnormal results of two screening tests, we present a quantitative method for interpreting these guidelines. METHODS: We obtained ocular coherence tomography (OCT) scans of 100 patients who have been on HCQ for 5 years or more (patients) and 70 age-matched controls. Both groups had 10'2 visual field (VF) test. We used linear regression to determine the cut-off points for each of the eight Early Treatment of Diabetic Retinopathy Study (ETDRS) macular sectors for the VF and OCT. We calculated the probability of developing maculopathy using logistic regression. RESULTS: Mean patient age: 59.9 years, 85% females, no statistically significant age difference between the patients and the control groups. DIAGNOSIS: 64% rheumatoid arthritis, 14% Sjogren's syndrome, 16% systemic lupus and 6% various other rheumatology conditions. Mean duration of use was 6.3 years. Logistic regression results show strong negative correlation between the outer nuclear layer (ONL) volume and probability of toxicity. Goodness of fit was tested using Hosmer and Lemeshow test that indicates a high significance with a high P-value of 1. CONCLUSIONS: Combining the ONL volume reduction and VF retinal sensitivity reduction per each of the eight ETDRS macular sectors provides an accurate and objective way of diagnosing HCQ maculopathy, this helps busy eye units establishing an optometrist-led or virtual service because it is independent of the assessor's level of experience.

Evaluation and calibration of a binocular infrared pupillometer for measuring relative afferent pupillary defect.

BACKGROUND: Binocular infrared pupillometry allows an estimate of the relative afferent pupillary defect (RAPD), designated the pupillometric RAPD (pRAPD). We calibrated the pRAPD of a commercially available pupillometer against neutral density filters (NDFs) of known attenuation. The performance of the pupillometer using its own proprietary algorithm is assessed and compared to that of alternative algorithms. METHODS: The pRAPDs of 50 healthy volunteers were measured with each of 4 filters of known attenuation: 0.0, 0.3, 0.6, and 0.9 log units, positioned unilaterally in the light stimulus pathway. The filter values were plotted against the pupillometer output, and the slope and intercept were used to determine a calibration factor. Corrected pRAPD results were used to assess physiological ranges of pRAPD. The sensitivity and specificity to 0.3 log unit differences between increasing filter densities using receiver operator characteristic (ROC) curves. RESULTS: The calibrated physiological pRAPD ranged from 0 to 0.22 log units. The area under the ROC curve for detecting unilateral simulated pRAPD of 0.3 log units, the simulated disease progression from 0.3 to 0.6 log units, and a further progression from 0.6 to 0.9 log units by NDFs was 0.99 (95% confidence interval [CI], 0.95-1.00), 0.86 (95% CI, 0.78-0.92), and 0.79 (95% CI, 0.70-0.87), respectively. The optimum discrimination was for detecting a unilateral simulated pRAPD of 0.3 log units; sensitivity and specificity was 98% (95% CI, 88%-99%). CONCLUSION: The commercially available pupillometer detects the RAPD induced by the NDFs with high sensitivity and specificity. The results suggest that it is best for detecting unilateral early disease but potentially useful for assessing progression of disease.

Improving the prediction accuracy of the SRK/T formula: the T2 formula.

PURPOSE: To investigate the causes of nonphysiologic behavior of the SRK/T formula, assess their clinical significance, and develop and evaluate solutions. SETTING: Two NHS ophthalmology departments, United Kingdom, and a private practice, United States. DESIGN: Evaluation of technology. METHODS: The individual steps of the SRK/T formula were examined for nonphysiologic behavior, and the clinical significance of behaviors was assessed with reference to a database of biometry and refractive outcomes in 11 189 eyes. The full data set was divided into 2 subsets, the first to develop solutions to nonphysiologic behavior of the SRK/T formula and the second to evaluate their performance. RESULTS: The SRK/T formula showed nonphysiologic behavior in the calculation of corrected axial length and corneal height. Although the former is of little clinical significance, the latter showed a systematic error that contributes to inaccurate intraocular lens (IOL) power prediction. The T2 formula was developed using a regression formula for corneal height derived from the development subset. Comparison of the performance of the T2 and SRK/T formulas using the evaluation subset showed significant improvement in the mean absolute error with the T2 formula (0.3064 diopter [D] versus 0.3229 D; P<.0001). On average, the prediction error with the T2 formula was 9.7% less than with the SRK/T formula, with significantly higher proportions of eyes within ±0.50 D of target (P<.0001). CONCLUSIONS: The SRK/T formula has nonphysiologic behavior that contributes to IOL power prediction errors. A modification to the formula algorithm, the T2 formula, can be directly substituted for SRK/T, resulting in significantly improved prediction accuracy.

Estimation of true corneal power after keratorefractive surgery in eyes requiring cataract surgery: BESSt formula.

PURPOSE: To describe a new formula, BESSt, to estimate true corneal power after keratorefractive surgery in eyes requiring cataract surgery. SETTING: Moorfields Eye Hospital, London, United Kingdom. METHODS: The BESSt formula, based on the Gaussian optics formula, was developed using data from 143 eyes that had keratorefractive surgery. The formula takes into account anterior and posterior corneal radii and pachymetry (Pentacam, Oculus) and does not require pre-keratorefractive surgery information. A software program was developed (BESSt Corneal Power Calculator), and corneal power was calculated in 13 eyes that had keratorefractive surgery and required cataract surgery. RESULTS: In the eyes having phacoemulsification, target refractions calculated with the BESSt formula were statistically significantly closer to the postoperative manifest refraction (mean deviation 0.08 diopters [D] +/- 0.62 [SD]) than those calculated with other methods as follows: history technique (-0.07 +/- 1.92 D; P = .05); history technique with double-K adjustment (0.13 +/- 2.39 D; P = .05); Holladay 2 with K-values estimated with the contact lens method (-0.76 +/- 1.36 D; P = .03); Holladay 2 with K-values from Atlas topographer (Humphrey) (-0.55 +/- 0.61 D; P<.01). Using the BESSt formula, 46% of eyes were within +/-0.50 D of the intended refraction and 100% were within +/-1.00 D. CONCLUSIONS: The BESSt formula was statistically significantly more accurate than the other techniques tested. Thus, it could significantly improve intraocular lens power calculation accuracy after keratorefractive surgery, especially when pre-refractive surgery data are unavailable.

Acute hydrops in keratoconus masquerading as acute corneal transplant rejection.

PURPOSE: To document 2 cases that developed acute hydrops corneae within the host tissue after corneal transplantation. Subsequent extension across the transplant-host interface led to edema of the transplanted tissue, with consequent misdiagnosis and treatment of acute transplant rejection. METHODS: The patients attended the Anterior Segment Service at Moorfields Eye Hospital, London, UK. RESULTS: Both patients had a reduction in vision, ocular discomfort, and stromal edema of the transplanted button and adjacent host cornea. No break in the Descemet membrane was noted in either case. CONCLUSION: It is important to differentiate hydrops of the host cornea from acute transplant rejection. This distinction will allow the judicious use of steroids and may allay patient anxiety about possible rejection.

Comparison of dynamic digital pupillometry and static measurements of pupil size in determining scotopic pupil size before refractive surgery.

PURPOSE: To compare 2 digital infrared pupillometers. SETTING: Anterior Segment Service, Moorfields Eye Hospital, London, England. METHODS: Eighty-eight eyes of 44 healthy subjects were recruited into the study. The scotopic pupil size was measured with 2 instruments, a digital pupillometer (Procyon) and a Hartman-Shack wavefront aberrometer (Visx WaveScan). Agreement between the instruments was assessed. RESULTS: There were 21 men and 23 women with a mean age 38.1 years +/- 9.2 (SD) (range 23 to 62 years). The mean scotopic pupil diameter was significantly larger with the WaveScan (6.61 +/- 0.92 mm) than with the Procyon pupillometer (6.40 +/- 0.90 mm), P<.001. CONCLUSIONS: There was good agreement in measurement of scotopic pupil size between the two instruments. The larger pupil diameter found with the Visx WaveScan than with the Procyon digital pupillometer probably represents the different illumination level used by each instrument, the effects of accommodation, and target fogging.

Unexpected posterior capsule rupture with unfolding silicone plate-haptic lenses.

PURPOSE: To evaluate the incidence, management, and long-term outcomes of unexpected posterior capsule rupture during injection of a C11UB plate-haptic silicone intraocular lens (IOL) with the Passport II system (both Bausch & Lomb Surgical). SETTING: Tertiary referral center and teaching hospital. METHODS: This retrospective analysis comprised 24 cases of posterior capsule rupture during plate-haptic silicone IOL injection. RESULTS: Over 6 months, a C11UB IOL was injected after phacoemulsification in 3446 cases, from which 24 patients were drawn. Thus, the rate of posterior capsule rupture was 0.70%. The median preoperative best corrected visual acuity was 6/48 (range 6/12 to light perception). The median best spectacle-corrected acuity at the time of discharge or the last visit was 6/9 (range 6/4 to 6/24). Twenty patients had improved acuity, 2 lost 1 Snellen line, and 2 had unchanged acuity. There were no postoperative complications in 13 patients (54%). Three patients required further surgery. Twenty-one patients were discharged after a mean of 32 weeks +/- 22 (SD); they required a mean of 5 +/- 4 visits. The remaining 3 continue to be followed because of their preoperative ocular comorbidity. CONCLUSIONS: Although no predisposing factor was identified, we believe the risk for posterior capsule rupture during IOL injection can be minimized by careful injection technique. In particular, if there is doubt about the integrity of the zonules, anterior capsule, or posterior capsule, a plate-haptic silicone IOL should not be injected. With the appropriate management, the final visual outcome was good.

Corneal swelling with overnight wear of scleral contact lenses.

PURPOSE: The management of some ocular surface diseases may be enhanced by the overnight wear of a rigid gas-permeable (RGP) scleral contact lens (ScCL). There is little information of the hypoxic effect of extended RGP ScCL wear. The authors measured the corneal swelling after overnight wear of a rigid gas permeable scleral contact lens in normal subjects. METHODS: Four subjects wore an RGP ScCL overnight in 1 eye only on 4 occasions. The following morning the lens was removed immediately before optical pachymetry, and the corneal thicknesses of both eyes were measured by a masked observer. On another four occasions optical pachymetry was performed, but the lens was not worn the previous night. For each subject the lens was of the same thickness. Specular microscopy was used to estimate endothelial cell density. RESULTS: There were no adverse effects, and subjects reported no difficulties in handling the lenses. Overnight wear of ScCL induced a variable amount of corneal swelling among subjects from 4.9% to 17.5%. The overnight swelling correlated strongly with endothelial cell density. CONCLUSIONS: Overnight wear of ScCL caused an increased degree of corneal swelling compared with daytime wear. This degree of swelling should not rule out overnight therapeutic ScCL wear if the disease process is deemed to be more damaging than the hypoxic effect of an RGP ScCL worn overnight. However, when used for refractive correction, they should not be left in overnight.

Effects of Gravity on ZBLAN Glass Crystallization.

The effects of gravity on the crystallization of ZrF(4)-BaF(2)-LaF(3)-AlF(3)-NaF glasses have been studied using the NASA KC-135 and a sounding rocket. Fibers and cylinders of ZBLAN glass were heated to the crystallization temperature in unit and reduced gravity. When processed in unit gravity the glass crystallized, but when processed in reduced gravity, crystallization was suppressed. A possible explanation involving shear thinning is presented to explain these results.