Search

The article presents a case study of 19-year-old woman with high-risk acute lymphoblastic leukaemia in first complete remission received an allogeneic bone marrow transplantation from her human leukocyte antigen identical sister after a conditioning regimen consisting of cyclophosphamide and total body irradiation. It mentions severe visual loss with visual acuity of 20/400 in both eyes in patient; and also mentions need for bone marrow transplantation who develop microangiopathy.

The uveitides are a collection of over 30 diseases characterised by intraocular inflammation. Previous work demonstrated that the agreement among uveitis experts on diagnosis was modest at best with some pairs of experts having chance alone agreement on selected diseases. The Standardisation of Uveitis Nomenclature (SUN) is a17‐year collaboration among experts in uveitis, ocular image grading, informatics, and machine learning to improve clinical and translational uveitis research. The SUN "Developing Classification Criteria for the Uveitides" project used a rigorous, multi‐phase approach to develop classification criteria for 25 of the most common uveitic diseases. The project's phases were: (1) informatics; (2) case collection; (3) case selection; (4) machine learning; and (5) consensus review and publication. The results were classification criteria with a high degree of accuracy (93.3%–99.3% depending on anatomic class of the uveitis), the goal of which is to form the basis for future uveitis research. [ABSTRACT FROM AUTHOR]

Purpose: To examine retinal vascular changes in the peripapillary and macular areas in patients with pituitary adenoma (PA) using optical coherence tomography angiography (OCTA). Methods: Cross‐sectional, retrospective study of 17 consecutive PA patients and 16 healthy subjects. All patients underwent a neuro‐ophthalmological examination to assess the presence of optic neuropathy (ON). Static automated perimetry (SAP), macular and optic disc structural OCT [retinal nerve fibre layer (RNFL) and ganglion cell complex (GCC) thicknesses] and OCTA were performed. Pituitary adenoma (PA) patients with ON were compared to those without ON and to healthy subjects. Results: Optic neuropathy (ON) was found in 16 eyes of nine PA patients. Peripapillary vessel density (ppVD) and macular vessel density (VD) in the superficial vascular plexus were significantly decreased in PA eyes with ON, compared to healthy eyes (45.21 ± 5.69 versus 50.52 ± 2.14% and 43.79 ± 5.03% versus 48.96 ± 2.94%, respectively). No significant difference in VD was observed in the macular deep vascular complex (DVC) between groups. Pituitary adenoma (PA) patients with ON had a mean ppVD reduction by 10.51% compared to healthy subjects. RNFL and GCC thicknesses were significantly reduced in PA eyes with ON compared to the other groups. Peripapillary VD (ppVD) significantly correlated with RNFL thickness and SAP mean deviation. Conclusions: Optical coherence tomography angiography showed a significant decrease in ppVD and superficial macular VD in PA eyes with ON compared to healthy eyes, according to RNFL and GCC thinning. Together with the absence of DVC alterations, it may provide further insights into neurovascular coupling. [ABSTRACT FROM AUTHOR]

Objective: Oral ulcers, the hallmark lesion of Behçet's disease (BD), can be disabling and resistant to conventional treatment, and there is a need for safe and effective treatment. We undertook this study to investigate the long‐term safety and efficacy of ustekinumab therapy for BD‐related oral ulcers that are resistant to colchicine. Methods: This multicenter, prospective, open‐label study included 30 patients who fulfilled the criteria of the International Study Group for BD and who were diagnosed as having active oral ulcers resistant to colchicine. Patients were treated subcutaneously with ustekinumab 90 mg at inclusion, at week 4, and then once every 12 weeks. Each patient was assessed longitudinally for the presence and number of oral ulcers, and median numbers of oral ulcers (with interquartile range [IQR]) were calculated. The primary efficacy end point was the proportion of patients at week 12 who experienced complete response, defined as having no oral ulcers. Results: The median number of oral ulcers per patient during ustekinumab therapy was significantly lower at week 12 compared to baseline (0 [IQR 0–1] versus 2 [IQR 2–3]; P < 0.0001). Complete response was achieved in 60.0% and 88.9% of patients at weeks 12 and 24, respectively. The median Behçet's Syndrome Activity Score (in which higher scores indicate more active disease) was significantly lower at weeks 12 and 24 (17.5 [IQR 10–42.5] and 10 [IQR 8–11], respectively) versus baseline (70 [IQR 50–70]; P < 0.0001). After a median follow‐up of 12 months (IQR 6–16 months), 26 patients (86.7%) were still receiving ustekinumab treatment. Reasons for ustekinumab discontinuation included BD flare (n = 3) and side effects (n = 1). Seven patients (23.3%) experienced adverse events, including headaches (n = 4) and asthenia (n = 2), with no serious side effects. Conclusion: Ustekinumab seems to be effective in treating BD‐related oral ulcers that are resistant to treatment with colchicine. [ABSTRACT FROM AUTHOR]

Purpose: The vast majority of ruptured aneurysms of the anterior communicating artery typically present with subarachnoid haemorrhage. Isolated visual complaints are very uncommon in this setting. We present an unusual case of a patient with an acute retrobulbar optic neuropathy, secondary to a ruptured anterior communicating artery aneurysm. Design: Observational case report. Methods: A 29-year-old woman was assessed for an acute, isolated unilateral optic neuropathy of unknown origin. Although an initial encephalic MRI was believed to be normal, an underlying ruptured anterior communicating artery aneurysm was eventually diagnosed when the patient became stuporous because of intracranial bleeding. Conclusions: Occurrence of an acute retrobulbar optic neuropathy may be the initial isolated sign related to a ruptured anterior communicating artery aneurysm, prompting an appropriate neuroradiological assessment. [ABSTRACT FROM AUTHOR]

To evaluate the duration of immunomodulatory therapy (corticosteroids, immunosuppressive drugs) with regard to the rate of relapses and clinical features (exudative retinal detachment or anterior uveitis) in inflammatory episodes of Vogt-Koyanagi-Harada disease. Data of all 42 patients diagnosed with acute uveitis associated with VKH disease during the period of January 2005 to December 2008 at the Pitié-Salpêtrière Hospital or at the Lariboisière Hospital in Paris, France were extracted by chart review. There were 31 patients (73.8%) with episodes of recurrence and were included in the study. At the first recurrence, 81% (13 patients) of exudative retinal detachments (ERD) were associated with an initial immunomodulatory treatment conducted ≤6 months (3.76 months ± 2.67). Conversely, an initial treatment duration of >6 months was associated with anterior uveitis signs for 66% of patients (eight patients) with anterior first recurrence (p = 0.0061). On second episode of recurrence, 75% of patients (three patients) who developed exudative retinal detachment had been managed by immunomodulatory therapy for ≤6 months with the total duration of immunomodulatory treatment ≤6 months during previous inflammatory episodes. Conversely, all 16 patients who presented anterior uveitis with additional manifestations (optic disc oedema, macular oedema, vitritis and/or 'Sunset glow' fundus) have been treated for more than 6 months or treated during the initial occurrence lasting more than 9 months (p = 0.0035). The duration of systemic corticosteroids (and/or immunosuppressive drug therapy) for ≤6 months at first and second recurrence was associated with features of further exudative retinal detachment instead of anterior uveitis in VKH disease. [ABSTRACT FROM AUTHOR]

To report long-term results of intravitreal (IVT) bevacizumab as first local treatment for choroidal neovascularization (CNV) secondary to uveitis. Files of patients receiving 1.25 mg/0.05 ml bevacizumab as primary local treatment for CNV were retrospectively reviewed. Main outcomes were change in best-corrected visual acuity (BCVA) and central foveolar thickness (CFT), treatment-related adverse events, and number and frequency of injections. Fifteen eyes from fifteen patients were included. Multifocal choroiditis and panuveitis were the diagnosis in seven, ampiginous choroiditis in two, and for six remaining, serpiginous choroiditis, sympathetic ophthalmia, Vogt-Koyanagi-Harada syndrome, punctuate inner choroidopathy, tuberculosis and idiopathic inflammation. In 13 eyes, neovascularization was subfoveal, and peripapillary in two. Intraocular inflammation was strictly controlled in all cases by the time of injections. BCVA improved from logMar 0.53 to logMar 0.29 in 12 eyes (80%), while CFT decreased from 239.06 to 195.2 μm in 13 (87%). Twelve eyes received more than one injection; mean number in this group was 4.25 (2-8), and frequency 1 every 12.97 weeks. There were no adverse events related to bevacizumab or the procedure. Median follow-up was 17.6 months (8-25). First-intention IVT bevacizumab for inflammatory CNV showed transient improvement in BCVA and CFT, in eyes under controlled inflammation. Reinjection was needed in most cases. Further work should conclude about safety related to repeated injections. [ABSTRACT FROM AUTHOR]

The article focuses on a study which investigated the efficacy and safety of interleukin-1 receptor antagonist anakinra in treating patients with neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic, cutaneous, articular syndrome (CINCA syndrome). The study included 10 patients with NOMID/CINCA syndrome who had been treated with anakinra. Study authors concluded that anakinra treatment is effective in NOMID/CINCA syndrome, but treatment has to be started before irreversible lesions develop.

Primary intraocular lymphoma (PIOL) is a rare presentation of lymphoma that is particularly difficult to recognize. In our institution, 36 cases of PIOL were diagnosed between March 1997 and July 2002. The recognition of lymphoma cells by cytology with or without immunophenotyping on slides generated a strong suspicion of the diagnosis in 34 of 36 cases. The diagnosis was confirmed by measurement of interleukin-10 (IL-10) in the vitreous humour or aqueous humour; high levels were observed in 35 of 36 cases, all were of B-cell origin. As expected, the only case with T-cell lymphoma had a very low level of IL-10. Furthermore, IL-10 levels excluded this diagnosis in two cases that were incorrectly suspected of PIOL after cytological examination. Finally, detection of clonality by polymerase chain reaction techniques, performed in 29 cases, represented a helpful tool in diagnosing PIOL as this approach definitively confirmed the diagnosis of B- or T-cell lymphoma in 17 cases. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discusses a study on the recurrences of late posterior segment manifestations in Vogt-Koyanagi-Harada (VKH) disease.

A letter to the editor is presented regarding the case report of the 29 year-old woman with idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis syndrome (IRVAN).