Development and validation of the Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI): a novel instrument to quantify organ damage in systemic sclerosis.

OBJECTIVE: We sought to develop the first Damage Index (DI) in systemic sclerosis (SSc). METHODS: The conceptual definition of 'damage' in SSc was determined through consensus by a working group of the Scleroderma Clinical Trials Consortium (SCTC). Systematic literature review and consultation with patient partners and non-rheumatologist experts produced a list of potential items for inclusion in the DI. These steps were used to reduce the items: (1) Expert members of the SCTC (n=331) were invited to rate the appropriateness of each item for inclusion, using a web-based survey. Items with >60% consensus were retained; (2) Using a prospectively acquired Australian cohort data set of 1568 patients, the univariable relationships between the remaining items and the endpoints of mortality and morbidity (Physical Component Summary score of the Short Form 36) were analysed, and items with p<0.10 were retained; (3) using multivariable regression analysis, coefficients were used to determine a weighted score for each item. The DI was externally validated in a Canadian cohort. RESULTS: Ninety-three (28.1%) complete survey responses were analysed; 58 of 83 items were retained. The univariable relationships with death and/or morbidity endpoints were statistically significant for 22 items, with one additional item forced into the multivariable model by experts due to clinical importance, to create a 23-item weighted SCTC DI (SCTC-DI). The SCTC-DI was predictive of morbidity and mortality in the external cohort. CONCLUSIONS: Through the combined use of consensus and data-driven methods, a 23-item SCTC-DI was developed and retrospectively validated.

Sensory impairment, use of community support services, and quality of life in aged care clients.

OBJECTIVE: To assess influences from visual or hearing impairment on use of community support services and health-related quality of life in aged care clients. METHOD: The authors sampled 284 frail elderly individuals presenting for assessment in Sydney, Australia. Moderate to severe visual impairment was defined as visual acuity <20/80 (better eye), and moderate to severe hearing loss as hearing thresholds >40 decibels (better ear). Community support services included home-delivered meals, home help, and community nurse visits. RESULTS: After adjusting for age, sex, and two or more comorbid conditions, moderate to severe visual impairment, but not moderate to severe hearing loss, was significantly associated with increased use of community services (adjusted odds ratio 2.8, 95% confidence interval = 1.0-7.8). CONCLUSION: Moderate to severe visual impairment was associated with an increased likelihood of community service utilization in this aged care client sample.

Visual manifestations of craniofrontonasal dysplasia.

In this sample of craniofrontonasal dysplasia, a 44.4% prevalence of visual impairment was observed, with more than half being due to potentially correctable causes of visual loss, including amblyopia and anisometropia. High prevalences of strabismus (88.9%) and V-pattern (55.5%) in craniofrontonasal dysplasia were also demonstrated. All three patients who underwent strabismus surgery showed improvement in ocular alignment postoperatively. This group needs regular eye examinations to assess for visual impairment and provide timely intervention for modifiable causes of visual loss.

Measures of disease status in systemic sclerosis: A systematic review.

OBJECTIVES: To identify and appraise measures of disease status in systemic sclerosis (SSc). METHODS: A systematic review of Medline (1966-2015), EMBASE (1974-2015), and Cochrane Library (inception-2015) was undertaken to identify indices of disease status in SSc. We focused on objective measures and excluded non-English articles. Measures were reviewed for content, whether they measured activity, damage and/or severity and whether they were validated according to the OMERACT filter. RESULTS: Of the 4558 articles retrieved through the search, we identified 58 articles for review. We found a further 44 articles through a search of the bibliography of relevant articles. We identified the following 10 "composite" (multi-organ) indices: two disease activity indices, six disease severity scales, and two combined response indices. There was no disease damage index for SSc. CONCLUSIONS: We identified a number of composite and organ-specific indices in SSc, incorporating mostly objective measures, developed to quantify disease activity, severity, and response in clinical trials. However, none of the indices was developed to exclusively quantify organ damage. Most of the existing indices require further validation according to the OMERACT filter. There is a need to develop and validate a disease damage index in SSc.

Are sensory and cognitive declines associated in older persons seeking aged care services? Findings from a pilot study.

INTRODUCTION: To assess the association between sensory and cognitive function, and the potential influence of visual function on cognitive function assessment, in a sample of clients accessing aged care services. MATERIALS AND METHODS: We recruited 260 non-institutionalised, frail, older individuals who sought aged care services. Visual acuity was assessed using a LogMAR chart and hearing function was measured using a portable pure-tone air conduction audiometer. Visual impairment was defined as visual acuity (VA) <6/12 (<39 letters read correctly in the better eye), moderate-to-severe hearing impairment as hearing thresholds >40 decibels (better ear) and cognitive impairment as Mini-Mental State Examination (MMSE) score <24. RESULTS: Vision and hearing assessments were randomly performed in 168 and 164 aged care clients, respectively. Visual acuity correlated weakly with MMSE scores, either including (r = 0.27, P <0.001) or excluding (r = 0.21, P = 0.006) vision-related MMSE items. After partialling out the effect of age, the association remained (r = 0.23, P = 0.013 including, or r = 0.18, P = 0.044 excluding vision-related items). No correlation was found between MMSE scores and hearing thresholds (r = -0.07, P = 0.375). After adjusting for age, sex and stroke, mean MMSE scores were lower in persons with visual impairment than those with normal vision (25.2 + 0.5 versus 26.8 + 0.4 including, or 18.2 + 0.5 versus 19.2 + 0.3 excluding vision-related items), but were similar between subjects with none or mild and those with moderate-to-severe hearing loss (26.3 + 0.4 versus 26.0 + 0.4). CONCLUSIONS: In this study sample, visual and cognitive functions were modestly associated, after excluding the influence of visual impairment on the MMSE assessment and adjusting for age. Hearing thresholds were not found to be associated with cognitive function.

Vision and self-rated health: longitudinal findings from an older Australian population.

PURPOSE: To assess temporal associations between vision and self-rated health. METHODS: We examined 3654 Australian residents (82.4% response) aged 49+ years in the Blue Mountains Eye Study, during 1992 to 1994, and re-examined 2334 in 1997 to 1999. Change in vision was defined if baseline and 5-year visual acuity (VA) changed by > or =10 letters (2 lines). Change in self-rated health was defined if overall health ranking (excellent, good, fair, poor) changed by > or =1 step. RESULTS: Of 2285 participants with data from both examinations, 59.8%, 23.9%, and 16.3% reported unchanged, declined, and improved self-rated health, respectively. Among persons whose self-rated health declined, the proportions with a decline in presenting VA, no change, or improvement were similar (25.2%, 23.8% and 22.5%, respectively, p for trend = 0.5). Among participants with decline, no change, or improvement in self-rated health, a similar magnitude in the age-sex adjusted mean VA decline (-1.0 to -2.7 letters) was observed. After adjusting for age, sex, hospitalizations, and incident chronic conditions, declining VA was not associated with increased decline in 5-year self-rated health (multivariate-adjusted odds ratio 0.99, 95% CI 0.75-1.30). CONCLUSION: There were no parallel 5-year changes in self-rated health and vision in this population.

Nanophthalmos in a Melanesian population.

PURPOSE: To characterize the ophthalmic features and causes of visual loss in a cohort of Melanesians living in New Caledonia with nanophthalmos. METHODS: In this observational study, axial length, visual acuity (VA), cycloplegic autorefraction were assessed and dilated fundus examination was performed. Visual impairment was defined as VA<6/12 in the better eye, hypermetropia as >+1.0 dioptre (D), astigmatism as >or=1.0 D and anisometropia as >or=1.0 D difference between both eyes. Unilateral amblyopia was defined as at least a two-line difference in VA between both eyes and bilateral amblyopia as VA<6/12 in both eyes which was not adequately explained by refractive error and macular folds. RESULTS: Seventeen community-dwelling participants (aged 1.1-45.3 years) with short axial length (range from 16.1 to 21.6 mm) were identified. Of the 17 subjects, 14 were found to have crowded optic discs, three with papillomacular folds, three with a papillomacular band and three with macular radial folds. Further, all subjects demonstrated bilateral hypermetropia (range from +1.3 D to +15.1 D). A high proportion of subjects had astigmatism (12) and anisometropia (nine) in at least one eye. Visual impairment was found in nine subjects: five bilateral and four unilateral. Causes of visual impairment included amblyopia (seven), ametropia (seven) and macular folds (two). Amblyopia was attributed to several factors, including hypermetropia, anisometropia, astigmatism and esotropia. CONCLUSIONS: In this sample of Melanesians with nanophthalmos, a spectrum of ophthalmic features that was consistent with intraocular crowding was found. Over half of the subjects were visually impaired, mostly due to amblyopia and ametropia. Further characterization of the underlying genetic cause of nanophthalmos in this cohort will be the focus of future studies.

Prevalence and causes of visual impairment in craniosynostotic syndromes.

BACKGROUND: To assess the prevalence and causes of visual impairment in patients with craniosynostotic syndromes of Apert, Crouzon, Pfeiffer, Saethre-Chotzen and craniofrontonasal dysplasia. METHODS: The medical records of patients who attended the Craniofacial Clinic at two large paediatric hospitals in Sydney, Australia between 1983 and 2004 were retrospectively reviewed. Presenting visual acuity (VA) was assessed using tests appropriate to age and cognition: 'fix and follow' in infants (<18 months old), Teller card acuity in preverbal children (18 months to less than 3 years old), Kay picture test or Sheridan-Gardiner test in children aged between 3 and less than 6 years and Snellen chart in those aged 6 years or older. Visual impairment was defined as the inability to fix and follow or presenting VA < 6/12 in the better eye. Amblyopia was defined as a two-line difference in VA between both eyes in the absence of an organic eye disease. RESULTS: Sixty-three patients with craniosynostotic syndromes were identified, of whom 55 had VA assessed at the first visit. Of these 55, 19 (35.5%) had bilateral visual impairment and 5 (9.1%) had unilateral visual impairment. Causes of visual impairment include amblyopia (16.7%), ametropia (25%), optic atrophy (16.7%) and exposure keratopathy (4.2%). Risk factors for amblyopia include strabismus (43.3%), astigmatism (> or =1.5 dioptres) (39.5%), hypermetropia (18.4%) and anisometropia (> or =1.5 dioptre difference between both eyes) (15.8%). Six of the 63 patients (9.5%) had papilloedema; those who were followed up showed gradual resolution of papilloedema following timely decompressive surgery. CONCLUSIONS: A high prevalence of visual impairment in patients with craniosynostotic syndromes was found, almost half of them due to potentially correctable causes, including amblyopia and ametropia. Optic atrophy remains an important cause of visual impairment. Further studies are needed to assess the timing and efficacy of intervention for modifiable causes of visual loss in craniosynostotic syndromes.

Sensory and cognitive association in older persons: findings from an older Australian population.

BACKGROUND: Explanations for associations found between sensory and cognitive function remain unclear. OBJECTIVE: To assess in an older Australian population: (1) the correlation between sensory and cognitive function across groups with a narrow age range; (2) any independent association between sensory and cognitive impairment. METHODS: We examined 3,509 non-institutionalised residents aged 50+ years in the second cross-sectional survey of the Blue Mountains Eye Study (1997-2000). Visual impairment was defined for best-corrected visual acuity (VA) < 6/12 in the better eye, moderate to severe hearing loss for hearing threshold > 40 dB (better ear), and likely cognitive impairment for Mini-Mental State Examination (MMSE) < 24 points. RESULTS: We found likely cognitive impairment in 3.3%, vision impairment in 2.7% and moderate to severe hearing loss in 10.5% of this population. Correlation between VA or hearing threshold and MMSE score increased with age. After adjusting for age, weak but significant correlation was evident in the normal ageing sample between vision and MMSE (r = 0.12 with vision items included and r = 0.11 with vision items excluded, both p < 0.0001), and between hearing thresholds and MMSE (r = -0.12, p < 0.0001). After adjusting for age, sex, education and history of stroke, persons with vision impairment had a lower mean MMSE score than those with normal vision, regardless of whether vision-related items were included (27.1 vs. 28.6, p < 0.001) or excluded (19.8 vs. 21.0, p < 0.001). Similarly, persons with moderate to severe hearing loss had a lower mean MMSE score than those without hearing loss (28.1 vs. 28.7, p < 0.001). Persons with likely cognitive impairment also had lower mean VA and higher mean hearing threshold than those without, after adjustment. CONCLUSIONS: We have documented an age-related correlation between sensory and cognitive function in a normal ageing sample. The association between sensory impairment and likely cognitive impairment remained significant after excluding vision-related MMSE items and adjusting for confounding factors. Our data suggest that age-related decline and the effect of visual impairment on the measurement of cognition only partly explain the association between sensory and cognitive impairments in older persons.

Eye care service utilization in older people seeking aged care.

BACKGROUND: To assess the need for, and the use of eye care services in older people seeking aged care. METHODS: In total, 188 people (69.1% of those eligible) aged 65+ years who were assessed for aged care provision at Westmead Hospital, Sydney, were recruited in 2003 and re-examined a year later. At baseline, presenting visual acuity (VA) was randomly assessed in half the participants. People with under-corrected refractive error (pinhole VA improved at least 10 letters in those with presenting VA <6/6), bilateral visual impairment (better eye VA <6/12), or self-reported visual problems, were recommended to have further assessment by eye care professionals. At follow up, information on utilization of eye care services in the past 12 months was collected and VA was assessed in all returned participants. RESULTS: Of the 188 baseline participants, 121 (70% of survivors) were revisited a year later. Overall, 90/121 participants (74%) had seen an eye care professional in the previous year. Of the 66 participants who were recommended to see an eye care professional, 42 (64%) were revisited and 37/42 (88%) complied with the recommendation. At revisit, bilateral visual impairment was found in 49/120 (41%). The proportion with bilateral visual impairment was lower in participants whose vision was assessed at baseline (35%) than in those whose vision was not assessed (47%, P = 0.17), and also lower among people who had visited an eye care professional during the previous 12 months (39%) than those who had not (45%, P = 0.57). CONCLUSIONS: This pilot study indicates a relatively high need for, and high utilization of eye care services in the subgroup of older people seeking aged care services.