The 2021 Portuguese Society of Ophthalmology joint guidelines with Paediatric Rheumatology on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis.

AIM: To develop the first Ophthalmology joint guidelines with Paediatric Rheumatology with recommendations on the screening, monitoring and medical treatment of juvenile idiopathic arthritis-associated uveitis (JIA-U), endorsed by the Portuguese Society of Ophthalmology (SPO). METHODS: A systematic literature review was conducted to include publications up to July 14th 2020, with no language restrictions, in order to include all the international position papers/guidelines concerning the medical management of JIA-U and randomised clinical trials assessing the efficacy and safety of medical treatment in this field. We searched through MEDLINE (PubMed), Scopus, Web of Science and Cochrane Library. The Delphi modified technique to generate consensus was used. Preliminary evidence statements were subject to an anonymous agreement assessment and discussion process using an online survey, followed by further discussion and update at a national meeting. A draft of the manuscript with all recommendations was then circulated among all participants and suggestions were incorporated. The final version was again circulated before publication. RESULTS: Twenty-six recommendations were developed focusing on the following topics: general management (3), screening and follow-up of uveitis (4), treatment (17) and health education in JIA-U among patients and families (2). CONCLUSION: These guidelines were designed to support the shared medical management of patients with JIA-U and emphasize the need for a multidisciplinary approach between Ophthalmology and Paediatric Rheumatology regarding the comprehensive care of JIA-U. We acknowledge that updating these recommendations will be warranted in the future, as more evidence becomes available. KEY-WORDS: juvenile idiopathic arthritis, uveitis, biological treatment, conventional immunosuppressive treatment, multidisciplinary management, guidelines, consensus, review, Delphi Technique.

Advanced Cases of Birdshot HLA-A29 Retinochoroiditis: Prevalence and Characteristics.

PURPOSE: To establish the prevalence, morphological, and functional characteristics and evolution of advanced cases of birdshot retinochoroiditis (BRC). METHODS: A retrospective review of all BRC cases seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland, with at least 3 years of follow-up since the onset of symptoms. The following parameters were evaluated: demographic characteristics, best-corrected visual acuity (BCVA), fundus photographs, fluorescein (FA) and indocyanine green (ICGA) angiography frames, and visual field evaluation from initial visit to last follow-up. RESULTS: Out of the 1920 patients with uveitis who were followed from 1995 until 2018, 32 (1.67%) were diagnosed with BRC; 20 of these 32 patients had sufficient data to be included. Three of these twenty patients (15.0%) had severe forms of the disease. Two patients had received insufficient treatment and one progressed despite attempted maximal therapy. The mean BCVA was 0.62 (± 0.42) at presentation, which was already at an advanced stage of the disease, and 0.51 (± 0.37) at last follow-up. The average visual field mean defect (VFMD) was 19.65 (± 4.71 dB) at presentation and 21.40 (± 2.45 dB) at last follow-up. Choroidal inflammatory activity monitored by ICGA decreased from 10.67 (± 9.18) at presentation to 6.67 (± 5.61) at last follow-up. FA revealed mild retinal vasculitis at 6.50 (± 4.76), which remained stable to 4.67 (± 3.20). EDI-OCT estimated choroidal thickness (EDI-OCT-CT) measurements were 246.36 (± 116.93 µm) at presentation and 231.30 (± 120.70 µm) at the last follow-up. CONCLUSIONS: In our setting, 15% of BRC cases had a severe course and suffered extensive irreversible chorioretinal damage, obviously due to insufficient treatment in two cases. This underlines the importance of correctly diagnosing BRC and initiating therapy as promptly as possible to avoid such outcomes in an otherwise possibly controllable disease.

Orbital solitary fibrous tumor: A painless mass after a dacryochystorhinostomy.

The solitary fibrous tumor (STF) is a rare spindle cell tumor that most often occurs in the pleura, mediastinum and other serosal sites, but it can be found anywhere. The authors present a case report of a 35-year-old male who was submitted to an eventless left external dacryocystorhinostomy (DCR) due to epiphora. During the surgery no tumors or anomalous tissues were visualized. Five months later the patient presented a painless mass at the lower medial quadrant of the left orbit, near the lacrimal fossa. A computed tomography, a magnetic resonance imaging and an orbital ultrasound were performed. A round, very vascularized and highly contrast enhanced mass was found. An anterior orbitotomy was done. Histological aspects and immunohistochemical markers were consistent with STF. The tumor was not visualized during the DCR. The authors think that the growth of the tumor and the weakening of the tissues in the medial canthus after the DCR allowed the tumor to move anteriorly within the orbit.

Low-grade "benign" birdshot retinochoroiditis: prevalence and characteristics.

PURPOSE: To establish the prevalence, morphologic and functional characteristics and evolution of mild birdshot retinochoroiditis (BRC). METHODS: Retrospective review of all BRC cases treated at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland, with at least 3 years of follow-up since the initial symptoms. Sub-Tenon's injection of triamcinolone was the first line of treatment if visual field changes were unilateral, with no additional treatment if visual field returned to normal. The percentage of patients who did not need systemic therapy was established, and the following parameters were evaluated: demographic characteristics, best-corrected visual acuity (BCVA), fundus photographs, fluorescein angiography (FA) and indocyanine green (ICGA) angiography frames and perimetry evaluation from initial visit to last follow-up. RESULTS: Twenty cases of BRC were included in this study. Three of these patients (15%) received only local periocular therapy and qualified as mild BRC, with a mean follow-up of 9.3 years. The BCVA was 0.89 ± 0.25 at presentation and 1.0 ± 0.39 at last follow-up. Average visual field mean defect was 5.05 ± 3.27 at presentation and 1.78 ± 0.95 at last follow-up. Depigmented fundus lesions remained stable from presentation to last follow-up. Choroidal inflammatory activity monitored by ICGA decreased from 11.66 ± 3.44 at presentation to 4.25 ± 2.87 at last follow-up. FA revealed mild retinal vasculitis (2.25 ± 3.20) which remained stable (2.00 ± 4.00). CONCLUSIONS: In our setting, 15% of BRC cases had a benign course, controlled with periocular treatment. It is important to identify such cases in order not to overtreat, exposing patients needlessly to potential side effects of aggressive and prolonged immunosuppressive treatment.

Laser flare photometry: a cost-effective method for early detection of endophthalmitis after intravitreal injection of anti-VEGF agents.

BACKGROUND: Intravitreal injection of anti-vascular endothelial growth factor agents is the most common intraocular procedure worldwide, inevitably causing more cases of post-injection endophthalmitis. The purpose of this study was to evaluate the utility of laser flare photometry in monitoring inflammation after intravitreal injection of anti-vascular endothelial growth factor agents, particularly to detect early stage post-injection endophthalmitis. A retrospective case review was performed of all patients who underwent flare assessment by laser flare photometry before and after intravitreal injection of bevacizumab or aflibercept at the Centre for Ophthalmic Specialized Care in Lausanne, Switzerland, between January 2015 and May 2018. The following data were retrieved: indication for intravitreal injection, medication administered, pre-injection and 72-h post-injection laser flare photometry values, and occurrence of post-injection endophthalmitis. A total of 736 injections were included in this study; 705 cases (95.8%) had a post-injection flare at 72 h ≤ 30 ph/ms, 29 cases (3.9%) had a post-injection flare at 72 h between > 30 and 50 ph/ms, and 2 cases (0.3%) had a post-injection flare at 72 h above > 50 ph/ms (664 and 742 ph/ms). These latter two cases were diagnosed as early-stage endophthalmitis. CONCLUSION: Laser flare photometry is a cost-effective method of screening for early stage post-injection endophthalmitis. Values > 50 ph/ms 72-h post-injection should prompt immediate evaluation by an ophthalmologist.

Blood in a Haab Stria.

We describe a case of an asymptomatic and spontaneous intracorneal hemorrhage in an adult with congenital glaucoma and blood collected in a Haab stria.

Primeiros 5 anos de Implementação do Programa de Rastreio de Retinopatia Diabética no Centro Hospitalar do Porto / First 5 years of Implementation of Diabetic Screening Program in Centro Hospitalar do Porto

Resumo Objetivo: Caracterizar a população de diabéticos referenciados à consulta de Oftalmologia do Centro Hospitalar do Porto (CHP) através do programa de rastreio da ARS Norte, avaliar este tipo de método de rastreio e perceber o impacto do mesmo na dinâmica de um Serviço de Oftalmologia. Métodos: Avaliação retrospectiva dos processos clínicos dos utentes diabéticos referenciados à consulta de Rastreio de Retinopatia Diabética (RD) do CHP através do programa de rastreio da ARS Norte, entre Janeiro de 2012 e Dezembro de 2016. As variáveis analisadas foram: dados demográficos, duração e tipo de Diabetes Mellitus (DM), seguimento prévio em consulta de oftalmologia, tipo de retinopatia diabética e orientação subsequente. Resultados: Dos 613 doentes diabéticos observados nesta consulta 2.6% tinham DM tipo 1 e 97.4% tinham DM tipo 2, com duração média da doença de 15.8 anos e 6.6% não apresentava lesões de RD em qualquer um dos olhos; lesões de RD não proliferativa estavam presentes em 90.7% dos doentes, sendo bilaterais em 83.7% dos casos e 2.7% apresentava lesões de RD proliferativa em ambos os olhos. No momento da consulta, 31.9% apresentava maculopatia, em pelo menos um dos olhos. 51,1% dos doentes tinha no momento da consulta indicação para tratamento; 15.2% integraram a consulta de diabetes ocular do CHP por necessidade de vigilância e 33.7% tiveram alta para reintegrarem este programa de rastreio. Conclusões: O programa de rastreio de RD apresenta-se como uma ferramenta útil e necessária no diagnóstico precoce e tratamento atempado das lesões de RD.

Abstract Purpose: To characterize the population of diabetics referred to the Ophthalmology Department of the Centro Hospitalar do Porto (CHP) from the screening program of ARS Norte, to evaluate this type of screening method and to perceive the its impact in the dynamic of an Ophthalmology Department. Methods: Retrospective evaluation of the clinical processes of diabetic patients referred to the CHP Diabetic Retinopathy (DR) Screening Consultation from the ARS Norte screening program, between January 2012 and December 2016. The variables analyzed were demographic data, duration and type of Diabetes Mellitus (DM), previous follow-up in ophthalmology consultation, type of diabetic retinopathy and subsequent orientation. Results: Of the 613 diabetic patients observed in our Department referred from this program, 2.6% had type 1 DM and 97.4% had type 2 DM, with a mean illness duration of 15.8 years. 6.6% had no lesions of DR in any of the eyes. Non-proliferative DR lesions were present in 90.7% of the patients, being bilateral in 83.7% of the cases and 2.7% had proliferative DR lesions in both eyes. At the time of the consultation, 31.9% had maculopathy in at least 1 of the eyes. 51.1% of the patients had indication for treatment at the time of the referral. 15.2% were integrated in ocular diabetes section of CHP due to the need for surveillance and 33.7% were discharged to reintegrate this screening program. Conclusions: The DR screening program is a useful and necessary tool for the early diagnosis and early treatment of DR lesions.

Glucose intolerance after chronic stress is related with downregulated PPAR-γ in adipose tissue.

BACKGROUND: Chronic stress is associated with increased risk of glucose intolerance and cardiovascular diseases, albeit through undefined mechanisms. With the aim of gaining insights into the latter, this study examined the metabolic profile of young adult male rats that were exposed to chronic unpredictable stress. METHODS: Young adult male rats were submitted to 4 weeks of chronic unpredictable stress and allowed to recover for 5 weeks. An extensive analysis including of morphologic, biochemical and molecular parameters was carried out both after chronic unpredictable stress and after recovery from stress. RESULTS: After 28 days of chronic unpredictable stress (CUS) the animals submitted to this protocol displayed less weight gain than control animals. After 5 weeks of recovery the weight gain rebounded to similar values of controls. In addition, following CUS, fasting insulin levels were increased and were accompanied by signs of impaired glucose tolerance and elevated serum corticosteroid levels. This biochemical profile persisted into the post-stress recovery period, despite the restoration of baseline corticosteroid levels. The mRNA expression levels of peroxisome proliferator-activated receptor (PPAR)-γ and lipocalin-2 in white adipose tissue were, respectively, down- and up-regulated. CONCLUSIONS: Reduction of PPAR-γ expression and generation of a pro-inflammatory environment by increased lipocalin-2 expression in white adipose tissue may contribute to stress-induced glucose intolerance.