Pub Date : 2024-09-16DOI: 10.1080/09273948.2024.2404077
Samy Zaher, Alexandra Kozyreff, Lucie Pothen, Halil Yildiz
Purpose: To review the clinical presentation and diagnostic workup of patients presenting with ocular sarcoidosis as initial manifestation of the disease and identify risk factors for disease recurrence.
Methods: All cases of sarcoidosis followed in our departments of Ophthalmology and Internal Medicine were retrospectively reviewed. Among 177 patients, 50 had ocular sarcoidosis as initial presentation of the disease. Clinical, biological, radiological, and treatment data were collected. Logistic regression analysis was conducted to identify risk factors for recurrence.
Results: Mean age of patients was 51 years, with an equal M/F ratio. Bilateral ocular involvement was present in 86% of cases, with granulomatous panuveitis being the most common manifestation (48%). Two-thirds of patients had extraocular symptoms related to the disease, mostly fatigue. The most frequently involved extraocular site was the supradiaphragmatic lymph nodes, identified in 84% of cases. CRP was normal in 68% of patients, while ACE and lysozyme were elevated in 19% and 64% of cases, respectively. Histopathological confirmation was obtained in 68% of patients. Female sex (odds ratio [OR] 6.66, 95% confidence interval [CI] 1.50-40.40, p = 0.021) and granulomatous panuveitis (OR 7.23, 95% CI 1.70-40.99, p = 0.013) were identified as significant risk factors for disease recurrence.
Conclusion: Ocular sarcoidosis is a common initial presentation of systemic sarcoidosis. Diagnosis should be suspected in the presence of bilateral granulomatous panuveitis. Chest CT scan or 18F-FDG PET/CT is mandatory for systemic workup and biopsy is necessary for definite diagnosis. Female sex and a panuveitis presentation are significantly associated with disease recurrence.
{"title":"Ocular Sarcoidosis as Initial Presentation of the Disease: Clinical Characteristics and Risk Factors Associated with Disease Recurrence.","authors":"Samy Zaher, Alexandra Kozyreff, Lucie Pothen, Halil Yildiz","doi":"10.1080/09273948.2024.2404077","DOIUrl":"https://doi.org/10.1080/09273948.2024.2404077","url":null,"abstract":"<p><strong>Purpose: </strong>To review the clinical presentation and diagnostic workup of patients presenting with ocular sarcoidosis as initial manifestation of the disease and identify risk factors for disease recurrence.</p><p><strong>Methods: </strong>All cases of sarcoidosis followed in our departments of Ophthalmology and Internal Medicine were retrospectively reviewed. Among 177 patients, 50 had ocular sarcoidosis as initial presentation of the disease. Clinical, biological, radiological, and treatment data were collected. Logistic regression analysis was conducted to identify risk factors for recurrence.</p><p><strong>Results: </strong>Mean age of patients was 51 years, with an equal M/F ratio. Bilateral ocular involvement was present in 86% of cases, with granulomatous panuveitis being the most common manifestation (48%). Two-thirds of patients had extraocular symptoms related to the disease, mostly fatigue. The most frequently involved extraocular site was the supradiaphragmatic lymph nodes, identified in 84% of cases. CRP was normal in 68% of patients, while ACE and lysozyme were elevated in 19% and 64% of cases, respectively. Histopathological confirmation was obtained in 68% of patients. Female sex (odds ratio [OR] 6.66, 95% confidence interval [CI] 1.50-40.40, <i>p</i> = 0.021) and granulomatous panuveitis (OR 7.23, 95% CI 1.70-40.99, <i>p</i> = 0.013) were identified as significant risk factors for disease recurrence.</p><p><strong>Conclusion: </strong>Ocular sarcoidosis is a common initial presentation of systemic sarcoidosis. Diagnosis should be suspected in the presence of bilateral granulomatous panuveitis. Chest CT scan or <sup>18</sup>F-FDG PET/CT is mandatory for systemic workup and biopsy is necessary for definite diagnosis. Female sex and a panuveitis presentation are significantly associated with disease recurrence.</p>","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142292375","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-16DOI: 10.1080/09273948.2024.2404515
Jacobo Emilio Enríquez-Fuentes, Jaime Lorenzo-Castro, Marco Antonio Pascual-Santiago, Ana María Colino-Gallardo, Pedro Arriola-Villalobos
Purpose: To report a case of bilateral panuveitis following Dostarlimab therapy for endometrial cancer.
Methods: Case report.
Results: A 73-year-old woman with a history of endometrial cancer, previously treated with Dostarlimab (an immune checkpoint inhibitor), presented to the ophthalmology emergency department with decreased visual acuity in her left eye. Her medical history included immune-mediated thyroid involvement, which led to the discontinuation of Dostarlimab. Subsequently, she developed pneumonitis and pancreatitis. On initial examination in the emergency room, she had poor vision (hand motion in the right eye and 20/200 on the Snellen chart in the left eye), an inflammatory reaction in the anterior chamber, with 1+ cells, small keratic precipitates and broad posterior synechiae in both eyes. Examination of the posterior pole revealed 3+ vitritis, which made it difficult to evaluate the retina. Treatment with oral and topical corticosteroids was initiated, resulting in an improvement in symptoms and the resolution of intraocular inflammation in several weeks.
Conclusion: Immune checkpoint inhibitors are used in the treatment of various types of cancer, with their efficacy demonstrated in different clinical trials. However, they are also associated with a range of adverse reactions. To date, no ocular adverse reactions related to the use of Dostarlimab have been published. A case of bilateral panuveitis potentially induced by Dostarlimab is described.
{"title":"Bilateral Panuveitis After Endometrial Cancer Treatment with Dostarlimab: A Case Report.","authors":"Jacobo Emilio Enríquez-Fuentes, Jaime Lorenzo-Castro, Marco Antonio Pascual-Santiago, Ana María Colino-Gallardo, Pedro Arriola-Villalobos","doi":"10.1080/09273948.2024.2404515","DOIUrl":"https://doi.org/10.1080/09273948.2024.2404515","url":null,"abstract":"<p><strong>Purpose: </strong>To report a case of bilateral panuveitis following Dostarlimab therapy for endometrial cancer.</p><p><strong>Methods: </strong>Case report.</p><p><strong>Results: </strong>A 73-year-old woman with a history of endometrial cancer, previously treated with Dostarlimab (an immune checkpoint inhibitor), presented to the ophthalmology emergency department with decreased visual acuity in her left eye. Her medical history included immune-mediated thyroid involvement, which led to the discontinuation of Dostarlimab. Subsequently, she developed pneumonitis and pancreatitis. On initial examination in the emergency room, she had poor vision (hand motion in the right eye and 20/200 on the Snellen chart in the left eye), an inflammatory reaction in the anterior chamber, with 1+ cells, small keratic precipitates and broad posterior synechiae in both eyes. Examination of the posterior pole revealed 3+ vitritis, which made it difficult to evaluate the retina. Treatment with oral and topical corticosteroids was initiated, resulting in an improvement in symptoms and the resolution of intraocular inflammation in several weeks.</p><p><strong>Conclusion: </strong>Immune checkpoint inhibitors are used in the treatment of various types of cancer, with their efficacy demonstrated in different clinical trials. However, they are also associated with a range of adverse reactions. To date, no ocular adverse reactions related to the use of Dostarlimab have been published. A case of bilateral panuveitis potentially induced by Dostarlimab is described.</p>","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142292374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-13DOI: 10.1080/09273948.2024.2400165
Samuel Hailemichael Henok,Menen Ayalew Shibeshi,Awot Yibrah,Zewdu Terefework,Getahun Tabor Emebet,John H Kempen
PURPOSEWe compared the distribution of the HLA-B27 allele among Ethiopian patients with acute anterior uveitis (AAU) and controls without that disease.METHODSThe clinical features of patients were collected from their medical records. HLA-B27 genotyping was performed for 64 patients, with AAU using the real-time polymerase chain reaction (RT-PCR), and the results were compared to those from a panel of 192 healthy, unrelated volunteer control participants (refraction patients and volunteers) free of signs of anterior uveitis. Other outcomes were assessed comparing HLA-B27 positive vs. negative patients.RESULTSThe histocompatibility antigen HLA-B27 was identified in 6 out of 64 (9.4%) AAU patients, compared with 1.0% in controls (Odds Ratio = 9.8, 95% CI (1.93, 50.01)). Sub-group analysis of the cases revealed that patients with HLA-B27 positivity had a higher incidence of ocular hypertension (Odds Ratio = 5.93, 95% CI (1.29, 27.2)).CONCLUSIONThe antigen HLA-B27 was represented ~10-fold more frequently in the patient group than in the control group. HLA-B27 allele distribution in both cases and controls is lower than in reports from Caucasian and Asian populations, but similar to South Africa.
目的我们比较了埃塞俄比亚急性前葡萄膜炎（AAU）患者和无此疾病的对照组中 HLA-B27 等位基因的分布情况。方法从病历中收集患者的临床特征。使用实时聚合酶链式反应（RT-PCR）对 64 名急性前葡萄膜炎患者进行了 HLA-B27 基因分型，并将结果与 192 名没有前葡萄膜炎症状的健康、无血缘关系的志愿者对照组（屈光不正患者和志愿者）的结果进行了比较。结果64例AAU患者中有6例（9.4%）发现了组织相容性抗原HLA-B27，而对照组中只有1.0%（Odds Ratio = 9.8, 95% CI (1.93, 50.01)）。对病例进行分组分析后发现，HLA-B27 阳性的患者眼压较高（Odds Ratio = 5.93，95% CI (1.29, 27.2)）。病例和对照组中的HLA-B27等位基因分布低于白种人和亚洲人，但与南非相似。
{"title":"Frequency of HLA-B27 Haplotype Among Patients with Acute Anterior Uveitis and Controls in Ethiopia.","authors":"Samuel Hailemichael Henok,Menen Ayalew Shibeshi,Awot Yibrah,Zewdu Terefework,Getahun Tabor Emebet,John H Kempen","doi":"10.1080/09273948.2024.2400165","DOIUrl":"https://doi.org/10.1080/09273948.2024.2400165","url":null,"abstract":"PURPOSEWe compared the distribution of the HLA-B27 allele among Ethiopian patients with acute anterior uveitis (AAU) and controls without that disease.METHODSThe clinical features of patients were collected from their medical records. HLA-B27 genotyping was performed for 64 patients, with AAU using the real-time polymerase chain reaction (RT-PCR), and the results were compared to those from a panel of 192 healthy, unrelated volunteer control participants (refraction patients and volunteers) free of signs of anterior uveitis. Other outcomes were assessed comparing HLA-B27 positive vs. negative patients.RESULTSThe histocompatibility antigen HLA-B27 was identified in 6 out of 64 (9.4%) AAU patients, compared with 1.0% in controls (Odds Ratio = 9.8, 95% CI (1.93, 50.01)). Sub-group analysis of the cases revealed that patients with HLA-B27 positivity had a higher incidence of ocular hypertension (Odds Ratio = 5.93, 95% CI (1.29, 27.2)).CONCLUSIONThe antigen HLA-B27 was represented ~10-fold more frequently in the patient group than in the control group. HLA-B27 allele distribution in both cases and controls is lower than in reports from Caucasian and Asian populations, but similar to South Africa.","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":3.3,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-13DOI: 10.1080/09273948.2024.2395290
Jing Shi,Qingfeng Cao,Changwei Huang,Xiang Luo
BACKGROUNDTo investigate the association of hypoxia-inducible factor-1α (HIF-1α), Janus tyrosine kinase-signal transducer and activator of transcription (JAK-STAT) gene polymorphisms with idiopathic scleritis in a Chinese Han population.METHODSTen single nucleotide polymorphisms (SNP) of HIF-1α, tyrosine kinase 2 (TYK2), signal transducer and activator of transcription 3 (STAT3), signal transducer and activator of transcription 4 (STAT4), and retinoid-related orphan nuclear receptors-γ (ROR-γ) were selected for this study. A total of 496 idiopathic scleritis patients and 1009 controls were genotyped by the MassARRAY platform and iPLEX Gold Genotyping Assay. The allele and genotype frequencies were analyzed by Chi-square test and Fisher's exact test. Stratified analyses were performed based on gender and anatomic locations of idiopathic scleritis.RESULTSThe frequencies of CC genotype (p = 6.18 × 10-4, Pc = 0.04, OR = 0.67,95%CI = 0.53-0.84) and C allele (p = 7.08 × 10-4, Pc = 0.04, OR = 0.71,95%CI = 0.58-0.87) for HIF-1α/rs2057482 were found significantly lower in idiopathic scleritis patients when compared to healthy controls. Stratified analysis depending on gender showed significant decreased frequencies of CC genotype (CC: p = 4.04 × 10-4, Pc = 0.02, OR = 0.54, 95%CI = 0.39-0.76) and C allele (C: p = 1.62 × 10-4, Pc = 0.01, OR = 0.58, 95%CI = 0.44-0.77) in male patients. Stratification analysis of rs2057482 according to location of scleritis did not show any significant difference between three subgroups and healthy controls.CONCLUSIONThis study showed association between polymorphism of HIF-1α/rs2057482 and susceptibility to idiopathic scleritis in Han Chinese male patients.
{"title":"The Association of HIF-1α/rs2057482 Polymorphism with Idiopathic Scleritis in a Chinese Han Population.","authors":"Jing Shi,Qingfeng Cao,Changwei Huang,Xiang Luo","doi":"10.1080/09273948.2024.2395290","DOIUrl":"https://doi.org/10.1080/09273948.2024.2395290","url":null,"abstract":"BACKGROUNDTo investigate the association of hypoxia-inducible factor-1α (HIF-1α), Janus tyrosine kinase-signal transducer and activator of transcription (JAK-STAT) gene polymorphisms with idiopathic scleritis in a Chinese Han population.METHODSTen single nucleotide polymorphisms (SNP) of HIF-1α, tyrosine kinase 2 (TYK2), signal transducer and activator of transcription 3 (STAT3), signal transducer and activator of transcription 4 (STAT4), and retinoid-related orphan nuclear receptors-γ (ROR-γ) were selected for this study. A total of 496 idiopathic scleritis patients and 1009 controls were genotyped by the MassARRAY platform and iPLEX Gold Genotyping Assay. The allele and genotype frequencies were analyzed by Chi-square test and Fisher's exact test. Stratified analyses were performed based on gender and anatomic locations of idiopathic scleritis.RESULTSThe frequencies of CC genotype (p = 6.18 × 10-4, Pc = 0.04, OR = 0.67,95%CI = 0.53-0.84) and C allele (p = 7.08 × 10-4, Pc = 0.04, OR = 0.71,95%CI = 0.58-0.87) for HIF-1α/rs2057482 were found significantly lower in idiopathic scleritis patients when compared to healthy controls. Stratified analysis depending on gender showed significant decreased frequencies of CC genotype (CC: p = 4.04 × 10-4, Pc = 0.02, OR = 0.54, 95%CI = 0.39-0.76) and C allele (C: p = 1.62 × 10-4, Pc = 0.01, OR = 0.58, 95%CI = 0.44-0.77) in male patients. Stratification analysis of rs2057482 according to location of scleritis did not show any significant difference between three subgroups and healthy controls.CONCLUSIONThis study showed association between polymorphism of HIF-1α/rs2057482 and susceptibility to idiopathic scleritis in Han Chinese male patients.","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":3.3,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142248696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
OBJECTIVETo evaluate visual impairment (VI) at presentation in pediatric uveitis cases in southern and eastern India.METHODSRetrospective analysis of 477 children across four tertiary centers (2015-2020). Best-documented visual acuity (ETDRS letters) in the better eye was used to categorize VI.RESULTSMean age at presentation was 11 ± 3.7 years; 43.8% were females, and 64.8% bilateral. Of 205 patients with complete investigations, 32.7% were undifferentiated uveitis, 31.7% infectious, 25.7% juvenile idiopathic arthritis-associated uveitis, and 10.2% other non-infectious uveitis. At presentation, 79.0% had none to mild VI, 12.6% moderate VI, and 8.4% severe VI to blindness. On generalized estimating equations analysis for individual eyes, prior oral steroids and older age were risk factors, while female gender, prior topical corticosteroids and immunosuppressive therapy were protective against moderate and/or severe VI.CONCLUSIONSOver a fifth of pediatric uveitis cases present with moderate/severe VI, especially in older age or with prior oral corticosteroid therapy.
{"title":"Visual Impairment at Presentation in Pediatric Uveitis: A Multicentric Study in 477 Children Across Southern and Eastern India.","authors":"Shabtab Nasir,Mohammed Hasnat Ali,Anamika Patel,Anup Kelgaonkar,Sameera Nayak,Mudit Tyagi,Somasheila Murthy,Soumyava Basu","doi":"10.1080/09273948.2024.2401134","DOIUrl":"https://doi.org/10.1080/09273948.2024.2401134","url":null,"abstract":"OBJECTIVETo evaluate visual impairment (VI) at presentation in pediatric uveitis cases in southern and eastern India.METHODSRetrospective analysis of 477 children across four tertiary centers (2015-2020). Best-documented visual acuity (ETDRS letters) in the better eye was used to categorize VI.RESULTSMean age at presentation was 11 ± 3.7 years; 43.8% were females, and 64.8% bilateral. Of 205 patients with complete investigations, 32.7% were undifferentiated uveitis, 31.7% infectious, 25.7% juvenile idiopathic arthritis-associated uveitis, and 10.2% other non-infectious uveitis. At presentation, 79.0% had none to mild VI, 12.6% moderate VI, and 8.4% severe VI to blindness. On generalized estimating equations analysis for individual eyes, prior oral steroids and older age were risk factors, while female gender, prior topical corticosteroids and immunosuppressive therapy were protective against moderate and/or severe VI.CONCLUSIONSOver a fifth of pediatric uveitis cases present with moderate/severe VI, especially in older age or with prior oral corticosteroid therapy.","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":3.3,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142214867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-11DOI: 10.1080/09273948.2024.2401139
Sarishka Desai,Paul A Gaudio
PURPOSETo report two patients who presented with scleritis associated with tubulointerstitial nephritis and uveitis syndrome (TINU).RESULTSA 13-year-old male initially presenting with unilateral panuveitis with scleritis in the fellow eye was ultimately diagnosed with TINU. A 33-year-old male previously diagnosed with TINU presented with recurrent bilateral episcleritis as well as bilateral scleritis eight years after his first episode of bilateral anterior uveitis. Both patients' scleritis responded to local corticosteroids and systemic immunosuppression.CONCLUSIONSScleritis should be considered among the ocular manifestations of TINU.
{"title":"Tubulointerstitial Nephritis and Uveitis Syndrome Presenting with Scleritis.","authors":"Sarishka Desai,Paul A Gaudio","doi":"10.1080/09273948.2024.2401139","DOIUrl":"https://doi.org/10.1080/09273948.2024.2401139","url":null,"abstract":"PURPOSETo report two patients who presented with scleritis associated with tubulointerstitial nephritis and uveitis syndrome (TINU).RESULTSA 13-year-old male initially presenting with unilateral panuveitis with scleritis in the fellow eye was ultimately diagnosed with TINU. A 33-year-old male previously diagnosed with TINU presented with recurrent bilateral episcleritis as well as bilateral scleritis eight years after his first episode of bilateral anterior uveitis. Both patients' scleritis responded to local corticosteroids and systemic immunosuppression.CONCLUSIONSScleritis should be considered among the ocular manifestations of TINU.","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":3.3,"publicationDate":"2024-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142214836","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-10DOI: 10.1080/09273948.2024.2401140
Pinar Çakar Ozdal,Ilknur Tugal-Tutkun
Intermediate uveitis is a relatively common form of intraocular inflammation that can be seen at any age. The vitreous is the primary site of inflammation; and the presence of snowballs or snowbank defines its subtype, pars planitis. Since it is a clinical diagnosis, it is important to recognize the typical clinical features and associated ocular complications. Recent advances in ocular imaging have made significant contributions to the clinical appraisal and studies of intermediate uveitis. This narrative review includes clinical signs, ocular complications, and ocular imaging of intermediate uveitis and pars planitis.
{"title":"Review for Disease of the Year Clinical Features and Ocular Imaging in Intermediate Uveitis and Pars Planitis.","authors":"Pinar Çakar Ozdal,Ilknur Tugal-Tutkun","doi":"10.1080/09273948.2024.2401140","DOIUrl":"https://doi.org/10.1080/09273948.2024.2401140","url":null,"abstract":"Intermediate uveitis is a relatively common form of intraocular inflammation that can be seen at any age. The vitreous is the primary site of inflammation; and the presence of snowballs or snowbank defines its subtype, pars planitis. Since it is a clinical diagnosis, it is important to recognize the typical clinical features and associated ocular complications. Recent advances in ocular imaging have made significant contributions to the clinical appraisal and studies of intermediate uveitis. This narrative review includes clinical signs, ocular complications, and ocular imaging of intermediate uveitis and pars planitis.","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":3.3,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142214865","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-10DOI: 10.1080/09273948.2024.2401146
Ilaria Maccora,Jared J Ebert,Grant S Schulert,Megan Quinlan-Waters,Alexandra Duell,Jennifer L Huggins,Cameron C Sapp,Tiffany Nguyen,Sunil K Srivastava,Arjun B Sood,Sheila T Angeles-Han
BACKGROUNDAutosomal dominant neovascular inflammatory vitreoretinopathy (NIV), formerly called "ADNIV," is a rare autoinflammatory condition mainly of adulthood caused by mutations in calcium-activated calpain-5 protease (CAPN5). Our aim is to report the treatment and visual outcomes of children newly diagnosed with NIV after systemic treatment.METHODSWe reviewed charts of patients ≤18 years old with CAPN5 gene mutation, ocular findings consistent with NIV, and treated with systemic immunosuppression for a minimum of 6 months. Treatment response was based on ophthalmic examination, ultra-widefield fluorescein-angiography (UWFFA), and optical coherence tomography (OCT).RESULTSEight children (16 eyes) were diagnosed with NIV at a median age of 14 (Range [R] 9-16) years, with a median follow-up of 18 months (R6-20). At diagnosis, one patient had impaired visual acuity (VA > 0.4), eight had vascular leakage, two had neovascularization, and three had macular edema. All responded to oral or local glucocorticoids but was not sustained. Systemic immunosuppression was started in seven patients with methotrexate and infliximab after a median time from diagnosis of 1.5 months (R0.5-2) and 3.2 months (R2.5-3.1), respectively. Infliximab was discontinued in all after a median time of 7 months (R3.5-10) for ineffectiveness, and 5/7 switched to tocilizumab and 1 to adalimumab. Five failed to respond (4 tocilizumab, 1 adalimumab) and one had a minimal response to tocilizumab.CONCLUSIONSWe report on the systemic treatment response of seven children with ADNIV treated with methotrexate, infliximab, and tocilizumab. None were able to control disease. Further studies are needed to understand long-term outcomes and the utility of systemic immunosuppression.
{"title":"Treatment and Visual Outcomes in Pediatric Patients with Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy: A Cohort Study.","authors":"Ilaria Maccora,Jared J Ebert,Grant S Schulert,Megan Quinlan-Waters,Alexandra Duell,Jennifer L Huggins,Cameron C Sapp,Tiffany Nguyen,Sunil K Srivastava,Arjun B Sood,Sheila T Angeles-Han","doi":"10.1080/09273948.2024.2401146","DOIUrl":"https://doi.org/10.1080/09273948.2024.2401146","url":null,"abstract":"BACKGROUNDAutosomal dominant neovascular inflammatory vitreoretinopathy (NIV), formerly called \"ADNIV,\" is a rare autoinflammatory condition mainly of adulthood caused by mutations in calcium-activated calpain-5 protease (CAPN5). Our aim is to report the treatment and visual outcomes of children newly diagnosed with NIV after systemic treatment.METHODSWe reviewed charts of patients ≤18 years old with CAPN5 gene mutation, ocular findings consistent with NIV, and treated with systemic immunosuppression for a minimum of 6 months. Treatment response was based on ophthalmic examination, ultra-widefield fluorescein-angiography (UWFFA), and optical coherence tomography (OCT).RESULTSEight children (16 eyes) were diagnosed with NIV at a median age of 14 (Range [R] 9-16) years, with a median follow-up of 18 months (R6-20). At diagnosis, one patient had impaired visual acuity (VA > 0.4), eight had vascular leakage, two had neovascularization, and three had macular edema. All responded to oral or local glucocorticoids but was not sustained. Systemic immunosuppression was started in seven patients with methotrexate and infliximab after a median time from diagnosis of 1.5 months (R0.5-2) and 3.2 months (R2.5-3.1), respectively. Infliximab was discontinued in all after a median time of 7 months (R3.5-10) for ineffectiveness, and 5/7 switched to tocilizumab and 1 to adalimumab. Five failed to respond (4 tocilizumab, 1 adalimumab) and one had a minimal response to tocilizumab.CONCLUSIONSWe report on the systemic treatment response of seven children with ADNIV treated with methotrexate, infliximab, and tocilizumab. None were able to control disease. Further studies are needed to understand long-term outcomes and the utility of systemic immunosuppression.","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":3.3,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142214866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-09-09DOI: 10.1080/09273948.2024.2392201
Alexandre Paiva, Ana Luiza Biancardi, Andre Curi
This study aims to describe the clinical and laboratory outcomes of patients with Acquired Immunodeficiency Syndrome (AIDS) presenting multifocal choroiditis secondary to Cryptococcus sp. infection...
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Pub Date : 2024-09-09DOI: 10.1080/09273948.2024.2400172
Eirini Kaisari,Jordan Loeliger,Jennifer E Thorne,Dominique Monnet,Linda Imikirene,Souhila Kecili,Antoine P Brézin
PURPOSEBirdshot chorioretinitis (BSCR) is an ocular HLA-related disease with variable clinical progression. We examine the quality of life (QOL) of BSCR individuals aged ≥80 years, providing insights into the long-term disease impact.METHODSWe utilized data from the CO-BIRD cohort (ClinicalTrials.gov Identifier: NCT05153057) conducted at Hôpital Cochin in Paris, France, focusing on BSCR patients aged ≥80. The main outcome was vision-related QOL using the National Eye Institute Visual Function Questionnaire-25 (NEI VFQ-25). We used Spearman correlation to explore the impact of better BCVA and MD on the composite score, and the Wilcoxon test to evaluate specific visual symptoms' effects.RESULTSThe study included 35 patients with a mean age of 83.9 ± 3.7 years, 74.3% of whom received systemic immunosuppression. The mean composite score was 58 ± 30, with a median of 75 (23-79). The lowest subscores were driving capacity (38 ± 38), mental health (49 ± 33), and role difficulties (50 ± 35), while the highest were for ocular pain (70 ± 25) and social function (70 ± 38). Decimal BCVA below 0.5 and MD below -6 dB were associated with lower subscores. BCVA and MD were strongly correlated with the composite score (R = 0.67). Symptoms of poor color and blurry vision were significantly associated with lower composite score (p < 0.005).CONCLUSIONMost BSCR patients over 80 in our cohort maintained sufficient vision for daily activities. The high standard deviation and wide range of VFQ-25 results reflect the heterogeneity of visual outcomes among elderly BSCR patients.
{"title":"Quality of Life in Patients with Birdshot Chorioretinitis Aged 80 and Older.","authors":"Eirini Kaisari,Jordan Loeliger,Jennifer E Thorne,Dominique Monnet,Linda Imikirene,Souhila Kecili,Antoine P Brézin","doi":"10.1080/09273948.2024.2400172","DOIUrl":"https://doi.org/10.1080/09273948.2024.2400172","url":null,"abstract":"PURPOSEBirdshot chorioretinitis (BSCR) is an ocular HLA-related disease with variable clinical progression. We examine the quality of life (QOL) of BSCR individuals aged ≥80 years, providing insights into the long-term disease impact.METHODSWe utilized data from the CO-BIRD cohort (ClinicalTrials.gov Identifier: NCT05153057) conducted at Hôpital Cochin in Paris, France, focusing on BSCR patients aged ≥80. The main outcome was vision-related QOL using the National Eye Institute Visual Function Questionnaire-25 (NEI VFQ-25). We used Spearman correlation to explore the impact of better BCVA and MD on the composite score, and the Wilcoxon test to evaluate specific visual symptoms' effects.RESULTSThe study included 35 patients with a mean age of 83.9 ± 3.7 years, 74.3% of whom received systemic immunosuppression. The mean composite score was 58 ± 30, with a median of 75 (23-79). The lowest subscores were driving capacity (38 ± 38), mental health (49 ± 33), and role difficulties (50 ± 35), while the highest were for ocular pain (70 ± 25) and social function (70 ± 38). Decimal BCVA below 0.5 and MD below -6 dB were associated with lower subscores. BCVA and MD were strongly correlated with the composite score (R = 0.67). Symptoms of poor color and blurry vision were significantly associated with lower composite score (p < 0.005).CONCLUSIONMost BSCR patients over 80 in our cohort maintained sufficient vision for daily activities. The high standard deviation and wide range of VFQ-25 results reflect the heterogeneity of visual outcomes among elderly BSCR patients.","PeriodicalId":19406,"journal":{"name":"Ocular Immunology and Inflammation","volume":null,"pages":null},"PeriodicalIF":3.3,"publicationDate":"2024-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142214868","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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