Giant-cell arteritis typically involves extracranial branches of the aorta, such as the temporal and vertebral arteries. Temporal arteritis and polymyalgia rheumatica often coexist, and systemic manifestations of inflammation occur in virtually all forms of giant-cell arteritis. This article reviews the mechanisms that contribute to the causes of giant-cell arteritis, with an emphasis on immune-mediated injury to arteries.

/pdf/medium-and-large-vessel-vasculitis-23h48us9p3.pdf

Medium- and Large-Vessel Vasculitis

Retinopathy of prematurity (ROP) is a common blinding disease in children in the developed world despite current treatment, and is becoming increasingly prevalent in the developing world. ROP progresses in two phases. The first phase begins with delayed retinal vascular growth after birth and partial regression of existing vessels, followed by a second phase of hypoxia-induced pathological vessel growth. Two major risk factors of ROP are the use of oxygen and a decreased gestation period. Excessive oxygen contributes to ROP through regulation of vascular endothelial growth factor (VEGF). Suppression of VEGF by oxygen in phase I of ROP inhibits normal vessel growth, whereas elevated levels of VEGF induced by hypoxia in phase II of ROP precipitate pathological vessel proliferation. Insulin-like growth factor 1 (IGF-1) is a critical non-oxygen-regulated factor in ROP. We have found that serum levels of IGF-1 in premature babies directly correlate with the severity of clinical ROP. IGF-1 acts indirectly as a permissive factor by allowing maximal VEGF stimulation of vessel growth. Lack of IGF-1 in preterm infants prevents normal retinal vascular growth in phase I of ROP, despite the presence of VEGF. As infants mature, rising levels of IGF-1 in phase II of ROP allows VEGF stimulated pathological neovascularization. These findings suggest that restoration of IGF-1 to normal levels might be useful in preventing ROP in preterm infants.

/pdf/retinopathy-of-prematurity-3a0x9e69fz.pdf

Retinopathy of Prematurity

Aim—To determine whether diVering policies with regard to the control of oxygen saturation have any impact on the number of babies who develop retinopathy of prematurity and the number surviving with or without signs of cerebral palsy at one year. Methods—An examination of the case notes of all the 295 babies who survived infancy after delivery before 28 weeks gestation in the north of England in 1990‐ 1994. Results—Babies given enough supplemental oxygen to maintain an oxygen saturation of 88‐98%, as measured by pulse oximetry,for at least the first 8 weeks of life developed retinopathy of prematurity severe enough to be treated with cryotherapy four times as often as babies only given enough oxygen to maintain an oxygen saturation of 70‐90% (27.2% v 6.2%). Surviving babies were also ventilated longer (31.4 v 13.9 days), more likely to be in oxygen at a postmenstrual age of 36 weeks (46% v 18 %), and more likely to have a weight below the third centile at discharge (45% v 17%). There was no diVerence in the proportion who survived infancy (53% v 52%) or who later developed cerebral palsy (17% v 15%). The lowest incidence of retinopathy in the study was associated with a policy that made little use of arterial lines. Conclusions—Attempts to keep oxygen saturation at a normal “physiological” level may do more harm than good in babies of less than 28 weeks gestation. (Arch Dis Child Fetal Neonatal Ed 2001;84:F106‐F110)

https://fn.bmj.com/content/fetalneonatal/84/2/F106.full.pdf

Pulse oximetry, severe retinopathy, and outcome at one year in babies of less than 28 weeks gestation

Objective To provide evidence-based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as exemplars of large vessel vasculitis. Methods Clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for GCA and TAK (27 for GCA, 27 for TAK). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to rate the quality of the evidence. Recommendations were developed by the Voting Panel, comprising adult and pediatric rheumatologists and patients. Each recommendation required ≥70% consensus among the Voting Panel. Results We present 22 recommendations and 2 ungraded position statements for GCA, and 20 recommendations and 1 ungraded position statement for TAK. These recommendations and statements address clinical questions relating to the use of diagnostic testing, including imaging, treatments, and surgical interventions in GCA and TAK. Recommendations for GCA include support for the use of glucocorticoid-sparing immunosuppressive agents and the use of imaging to identify large vessel involvement. Recommendations for TAK include the use of nonglucocorticoid immunosuppressive agents with glucocorticoids as initial therapy. There were only 2 strong recommendations; the remaining recommendations were conditional due to the low quality of evidence available for most PICO questions. Conclusion These recommendations provide guidance regarding the evaluation and management of patients with GCA and TAK, including diagnostic strategies, use of pharmacologic agents, and surgical interventions.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/acr.24632

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis

The incidence, pathogenesis and treatment of cystoid macular edema following cataract surgery.

Aim: To describe the development and application of a novel scoring system for grading the severity of intermittent distance exotropia (IDEX) and its potential application as an intervention criterion for surgical intervention. Methods: The Newcastle Control Score (NCS) for IDEX was developed by incorporating both subjective (home control) and objective (clinic control) criteria into a scale to grade severity. The score structure described was evaluated for interobserver and test-retest reliability. To determine an optimal score threshold for surgical intervention, 170 cases of IDEX were scored retrospectively. Cure rates for surgical and non-surgical cases were then compared according to preoperative or presenting scores. Results: Interobserver and test-test reliability were good (r = 0.82 and r = 0.89 respectively). Total cure rate with surgery was 54% and without surgery 18% (χ2 = 23.093, df = 1, p Conclusions: The NCS is a reliable method for grading the severity of IDEX and aids decisions regarding intervention. Patients with a score of 3 or more are unlikely to attain a cure without surgery.

https://bjo.bmj.com/content/bjophthalmol/88/2/233.full.pdf

The Newcastle Control Score: a new method of grading the severity of intermittent distance exotropia

Forty-six cases of vitreous loss during planned extracapsular cataract extraction salvaged by anterior vitrectomy and primary anterior chamber lens implantation were compared in a retrospective cohort study with 92 individually matched controls. The postoperative visual outcome for cases with vitreous loss was significantly poorer than that for controls even after adjustment for confounding variables. Part of this visual morbidity was explained by cystoid macular oedema and retinal detachment, but even after adjusting for these two complications visual outcome remained worse among cases than controls. There was a significantly higher incidence of clinically apparent cystoid macular oedema amongst cases compared with controls (20% versus 1%). Two retinal detachments occurred among the cases but this difference in incidence was not statistically significant.

/pdf/vitreous-loss-in-planned-extracapsular-cataract-extraction-jnnv7kaifb.pdf

Vitreous loss in planned extracapsular cataract extraction does lead to a poorer visual outcome.

The Newcastle Control Score (NCS) has been shown to be a reliable, clinically sensitive method for grading the severity of childhood intermittent exotropia (X(T)).1 It incorporates subjective (home control) and objective (clinic control at near and in the distance) criteria, and uses modified descriptions of control as outlined by Rosenbaum and Santiago.2 The home control section asks the parent/guardian …

Grading the severity of intermittent distance exotropia: the revised Newcastle Control Score

Aim: To evaluate the use of the Newcastle Control Score (NCS) in the management of intermittent exotropia (X(T)). Participants and methods: Children aged Results: Baseline data were obtained on 272 children and follow-up data on 157. Mean (SD) age was 4 (1.9) years. Complete NCSs were obtained for all except one child at baseline, and all children at follow-up. At baseline, total NCS and the home control component were correlated with near stereo (r = –0.22, p Conclusion: The NCS is a useful measure of the clinical severity of X(T), can be used to serially assess improvement or deterioration and is a useful tool for the management of these patients.

/pdf/the-use-of-the-newcastle-control-score-in-the-management-of-3ifa9ui04v.pdf

The use of the Newcastle Control Score in the management of intermittent exotropia.

Ocular outcome was assessed in a 2 year cohort of all children born before 32 weeks gestation within a geographically defined population of approximately 3 million. Five hundred and sixty-five children were born within the study period ; 558 children (98.8% of the study group) were assessed at approximately 2 years old. There was a high incidence of abnormalities, particularly strabismus (70, 12.5%), cortical visual loss (7, 1.3%), sequelae of retinopathy of prematurity (16, 2.9%) and other significant refractive errors (69, 12.7%). These data provide a recent estimate of gestational age-specific prognosis and suggest that follow-up yields a significant proportion of children with visual abnormalities who could benefit from treatment.

Ocular outcome in children born before 32 weeks gestation.

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