RESUMO
INTRODUCTION: There is a scarcity of information available on clinical and laboratory features of adult-onset idiopathic autoimmune uveitis. Therefore, we conducted a single centre descriptive cross-sectional study. Patients and Methods. A chart review of all patients with idiopathic autoimmune uveitis with onset after 18 years of age who were referred to the rheumatology department between January 2017 and December 2018 was performed. Their clinical features, demographic features, and HLA-B genotypes were documented and described. RESULTS: Out of 210 patients referred to rheumatology, 66 were found to have uveitis, and 16 of these had an adult-onset idiopathic autoimmune uveitis. Apart from a slight female preponderance (62.5%), our patients were characterized by a high proportion of panuveitis (4 out of 16, i.e., 25%). There was an increased frequency of occurrence of synechiae (5 out of 16, i.e., 31.3%), retinal vasculitis (4 out of 16, i.e., 25%), optic disc edema (3 out of 16, i.e., 18.8%), and cystoid macular edema (seen in 2 patients, i.e., 12.5%). These features correlated with the anatomical subtypes. Retinal vasculitis and optic disc edema present in three fourth of all panuveitis cases were the most prominent features. The odds of finding HLA-B∗35 in retinal vasculitis were 33 times higher than odds of finding it in idiopathic autoimmune uveitis patients not having retinal vasculitis (OR 33; 95% CI 1.6-698). CONCLUSION: Idiopathic autoimmune uveitis in our patients is characterized by a high frequency of panuveitis and retinal vasculitis, and complications with a probable association between HLA-B∗35 and retinal vasculitis.
Assuntos
Doenças Autoimunes/patologia , Antígenos HLA-B , Uveíte/patologia , Adulto , Doenças Autoimunes/complicações , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/imunologia , Estudos Transversais , Feminino , Antígenos HLA-B/imunologia , Humanos , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Pan-Uveíte/complicações , Pan-Uveíte/epidemiologia , Pan-Uveíte/imunologia , Pan-Uveíte/patologia , Papiledema/etiologia , Papiledema/patologia , Vasculite Retiniana/etiologia , Vasculite Retiniana/patologia , Estudos Retrospectivos , Centros de Atenção Terciária/estatística & dados numéricos , Uveíte/complicações , Uveíte/epidemiologia , Uveíte/imunologia , Adulto JovemRESUMO
To study the correlation of OCT parameters including central subfield macular thickness (CSMT), peripapillary retinal thickness (PRT), and peripapillary retinal nerve fiber layer thickness (PNFLT) with fluorescein angiography (FA) in evaluation of inflammatory activity in Behcet's retinal vasculitis. In this case-series, concurrent FA and OCT were performed. A scoring system was devised for FA. PNFLT in 3.4-mm-diameter circle as well as PRT in doughnut shaped regions between the 1-mm- and 2.2-mm-diameter and between the 2.2-mm- and 3.45-mm-diameter circles was measured. The correlation of FA and OCT parameters was analyzed. A total of 105 sets of FA from 28 eyes (15 patients) were reviewed. Four (26.6%) were female and mean age was 31.6 ± 8.49 years. Each micron increase in CSMT, PRT2.2, PRT3.45, and PNFLT, caused a rise of 0.018 (95% CI 0.008-0.027, P < 0.001, r = 0.413), 0.053 (95% CI 0.035-0.070, P < 0.001, r = 0.443), 0.086 (95% CI 0.065-0.108, P < 0.001, r = 0.707), and 0.185 (95% CI 0.152-to 0.218, P < 0.001, r = 0.850) unit in FA score, respectively. Parameters having significant correlation with angiographic inflammatory activity, were CSMT, PRT2.2, PRT3.45 and RNFLT. Those with the strongest correlation, PRT3.45 and PNFLT, may be considered as quantitative non-invasive alternatives to FA for monitoring Behcet's retinal vasculitis.
Assuntos
Síndrome de Behçet/patologia , Angiofluoresceinografia/métodos , Monitorização Fisiológica/métodos , Fibras Nervosas/patologia , Vasculite Retiniana/patologia , Vasos Retinianos/patologia , Tomografia de Coerência Óptica/métodos , Adolescente , Adulto , Síndrome de Behçet/diagnóstico por imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Vasculite Retiniana/diagnóstico por imagem , Vasos Retinianos/diagnóstico por imagem , Adulto JovemRESUMO
Zika virus (ZIKV), a mosquito-borne flavivirus, can cause severe eye disease and even blindness in newborns. However, ZIKV-induced retinal lesions have not been studied in a comprehensive way, mechanisms of ZIKV-induced retinal abnormalities are unknown, and no therapeutic intervention is available to treat or minimize the degree of vision loss in patients. Here, we developed a novel mouse model of ZIKV infection to evaluate its impact on retinal structure. ZIKV (20 plaque-forming units) was inoculated into neonatal wild type C57BL/6J mice at postnatal day (P) 0 subcutaneously. Retinas of infected mice and age-matched controls were collected at various ages, and retinal structural alterations were analyzed. We found that ZIKV induced progressive neuronal and vascular damage and retinal inflammation starting from P8. ZIKV-infected retina exhibited dramatically decreased thickness with loss of neurons, initial neovascular tufts followed by vessel dilation and degeneration, increased microglia and leukocyte recruitment and activation, degeneration of astrocyte network and gliosis. The above changes may involve inflammation and endoplasmic reticulum stress-mediated cell apoptosis and necroptosis. Moreover, we evaluated the efficacy of preclinical drugs and the safety of ZIKV vaccine candidate in this mouse model. We found that ZIKV-induced retinal abnormalities could be blocked by a selective flavivirus inhibitor NITD008 and a live-attenuated ZIKV vaccine candidate could potentially induce retinal abnormalities. Overall, we established a novel mouse model and provide a direct causative link between ZIKV and retinal lesion in vivo, which warrants further investigation of the underlying mechanisms of ZIKV-induced retinopathy and the development of effective therapeutics.
Assuntos
Retina/crescimento & desenvolvimento , Retina/virologia , Degeneração Retiniana/patologia , Degeneração Retiniana/virologia , Infecção por Zika virus/patologia , Zika virus , Animais , Animais Recém-Nascidos , Camundongos , Camundongos Endogâmicos C57BL , Vasculite Retiniana/patologia , Vasculite Retiniana/virologia , Vasos Retinianos/patologia , Vasos Retinianos/virologia , Zika virus/isolamento & purificaçãoRESUMO
Background: Coats-like retinal vasculopathy in retinitis pigmentosa (RP) is rare. This study describes its clinical spectrum, management outcomes and genetic associations in patients with autosomal recessive RP (arRP).Materials and methods: Retrospective review of ophthalmic, multimodal imaging, genetic findings and treatment outcomes of arRP patients who developed Coats-like features. Identification of patients included searching a retinal dystrophy registry of 798 patients.Results: Ten eyes of six patients with arRP (4 males, 2 females, mean age 33 years) demonstrated Coats-like features, namely inferotemporal peripheral retinal telangiectasis combined with unilateral inferotemporal vasoproliferative tumor (VPT) in 4 eyes. Exudative retinal detachment (ERD) developed in five eyes of which four had VPT. Ablation of the vasculopathy using retinal laser photocoagulation and/or cryotherapy in eight eyes, allowed ERD and/or lipid exudation to decrease in seven eyes despite incomplete vasculopathy regression. Additional intravitreal triamcinolone acetonide injection in one eye failed to regress the ERD and associated VPT. Observation in one eye caused increased exudation. Six mutations, including three novel mutations, were found in CRB1, CNGB1, RPGR, and TULP1.Conclusions: Coats-like features in arRP range from retinal telangiectasis to VPTs with extensive ERD and occur predominantly in the inferotemporal retinal periphery. In addition to their classic association with CRB1 mutations, other genes are implicated. To the best of our knowledge, this is the first report describing CNGB1 mutations in Coats-like RP. Awareness of the vasculopathy spectrum is important, and timely ablation of the vasculopathy with long-term monitoring is recommended to prevent additional visual loss in RP patients.
Assuntos
Crioterapia/métodos , Proteínas do Olho/genética , Fotocoagulação a Laser/métodos , Descolamento Retiniano/cirurgia , Vasculite Retiniana/cirurgia , Retinose Pigmentar/complicações , Doenças Vasculares/cirurgia , Adulto , Exsudatos e Transudatos , Feminino , Genes Recessivos , Humanos , Masculino , Mutação , Descolamento Retiniano/patologia , Vasculite Retiniana/etiologia , Vasculite Retiniana/patologia , Estudos Retrospectivos , Doenças Vasculares/etiologia , Doenças Vasculares/patologiaRESUMO
ABSTRACT Wide-field angiography enables assessing peripheral areas with better quality and gives greater deep focus, which improves the image periphery. Some studies have proposed the usefulness of these angiographic systems in inflammatory diseases of the retina. However, few studies have evaluated this technique in Eales disease. We present a case series in which 5 eyes of 3 patients with Eales disease were evaluated by using retinal fluorescein angiography with 30º, 50º, and 150º lenses in a laser-scanning ophthalmoscope. These cases highlight the usefulness of wide-field fluorescein angiography in the diagnosis and follow-up of peripheral ischemic retinal areas in Eales disease, which enables better follow-up than possible with conventional fluorescein angiography images.
RESUMO A angiografia de campo amplo permite avaliar áreas periféricas com melhor qualidade e proporciona maior foco profundo, melhorando a imagem da periferia. Alguns estudos têm proposto a utilidade desses sistemas angiográficos nas doenças inflamatórias da retina. No entanto, poucos estudos avaliaram esta técnica na doença de Eales. Apresentamos uma série de casos em que 5 olhos de 3 pacientes com doença de Eales foram avaliados usando angiografia de fluoresceína da retina com lentes de 30º, 50º e 150º em um oftalmoscópio de varredura a laser. Esses casos destacam a utilidade da angiografia com fluoresceína de campo amplo no diagnóstico e no acompanhamento das áreas isquêmicas periféricas da retina na doença de Eales, permitindo um melhor acompanhamento do o possível com imagens por angiofluoresceinografia convencional.
Assuntos
Humanos , Masculino , Feminino , Adulto , Adulto Jovem , Angiofluoresceinografia/métodos , Vasculite Retiniana/diagnóstico por imagem , Neovascularização Patológica/diagnóstico por imagem , Retina/diagnóstico por imagem , Fatores de Tempo , Acuidade Visual , Reprodutibilidade dos Testes , Seguimentos , Vasculite Retiniana/patologia , Vasculite Retiniana/terapia , Neovascularização Patológica/patologia , Neovascularização Patológica/terapiaRESUMO
Wide-field angiography enables assessing peripheral areas with better quality and gives greater deep focus, which improves the image periphery. Some studies have proposed the usefulness of these angiographic systems in inflammatory diseases of the retina. However, few studies have evaluated this technique in Eales disease. We present a case series in which 5 eyes of 3 patients with Eales disease were evaluated by using retinal fluorescein angiography with 30º, 50º, and 150º lenses in a laser-scanning ophthalmoscope. These cases highlight the usefulness of wide-field fluorescein angiography in the diagnosis and follow-up of peripheral ischemic retinal areas in Eales disease, which enables better follow-up than possible with conventional fluorescein angiography images.
Assuntos
Angiofluoresceinografia/métodos , Neovascularização Patológica/diagnóstico por imagem , Vasculite Retiniana/diagnóstico por imagem , Adulto , Feminino , Seguimentos , Humanos , Masculino , Neovascularização Patológica/patologia , Neovascularização Patológica/terapia , Reprodutibilidade dos Testes , Retina/diagnóstico por imagem , Vasculite Retiniana/patologia , Vasculite Retiniana/terapia , Fatores de Tempo , Acuidade Visual , Adulto JovemRESUMO
Purpose: To report occurrence of acute severe inflammation after surgical implantation of mycoplasma-infected induced pluripotent stem cell-derived RPE (iPS-RPE) cells into the eyes of healthy primates, and determine the immunopathological mechanisms of the inflammation. Methods: Ophthalmic allogeneic transplantation of iPS-RPE cells was performed in the subretina of major histocompatibility complex (MHC)-matched (two eyes) and MHC-mismatched (one eye) healthy cynomolgus monkeys. The clinical course after transplantation was observed using color fundus photography, fluorescence angiography, and optical coherence tomography. After the animals were killed at 1 month after surgery, eyeballs were removed and pathologically examined. Microorganisms were analyzed by PCR methods and BLAST analysis using preserved graft iPS-RPE cells and the recipients' vitreous humor. Mixed lymphocyte-RPE assay was performed on the mycoplasma-infected and noninfected iPS-RPE cells in vitro. Results: In tested eyes, abnormal findings were observed in the grafted retina 2 weeks after surgery. Here, we observed retinal vasculitis and hemorrhage, retinal detachment, and infiltration of inflammatory cells into the retina of the eyes. One month after surgery, animals were killed due to the severe immune responses observed. Using PCR methods, sequence analysis detected mycoplasma-DNA (Mycoplasma arginini species) in both the grafted RPE cells and the collected vitreous fluids of the monkeys. Mixed lymphocyte-RPE assay revealed that the infected iPS-RPE cells enhanced the proliferation of inflammatory cells in vitro. Conclusions: Transplantation of graft iPS-RPE cells contaminated with mycoplasma into the subretina caused severe ocular inflammation. Mycoplasma possesses the ability to cause immune responses in the host.
Assuntos
Transplante de Células/efeitos adversos , Infecções Oculares/microbiologia , Células-Tronco Pluripotentes Induzidas/citologia , Infecções por Mycoplasma/patologia , Mycoplasma/isolamento & purificação , Epitélio Pigmentado da Retina/transplante , Animais , Transplante de Células/métodos , DNA Forma C/análise , Modelos Animais de Doenças , Infecções Oculares/etiologia , Inflamação/patologia , Macaca fascicularis , Infecções por Mycoplasma/etiologia , Complicações Pós-Operatórias/microbiologia , Descolamento Retiniano/patologia , Epitélio Pigmentado da Retina/citologia , Epitélio Pigmentado da Retina/patologia , Vasculite Retiniana/patologiaRESUMO
PURPOSE: To evaluate vascular changes in patients with intermediate uveitis with or without retinal vasculitis using swept-source wide-field optical coherence tomography angiography (OCTA). METHODS: This is a prospective cross-sectional study. Consecutive patients with intermediate uveitis were evaluated using wide-field OCTA. Wide-field OCTA and en-face OCT images were analysed for the presence of capillary non-perfusion and reduced perfusion, disruption of ellipsoid zone, and abnormalities on en-face wide-field retinal thickness maps, respectively, and compared with fluorescein angiography (FA) findings in a subcohort. RESULTS: 164 eyes of 88 patients with intermediate uveitis were included. Areas of capillary non-perfusion and reduced perfusion were more frequently observed in the choroidal OCTA slab (33.3% and 49.4%), choriocapillaris (CC; 31.4% and 48%) and deep capillary plexus (DCP; 9.6% and 34.6%) than in the superficial capillary plexus (SCP; 5% and 26.3%), respectively. Intermediate uveitis with vasculitis presented more frequently with non-perfusion and hypoperfusion in the DCP (p=0.003 and p=0.05, respectively) and SCP (p=0.007 and p=0.005, respectively) than intermediate uveitis without vasculitis. Peripheral capillary leakage on FA correlated with the presence of perivascular, macular and generalised thickening on en-face wide-field thickness maps (p=0.007). Ischaemia on FA was significantly associated with non-perfusion on wide-field OCTA in SCP and DCP (p=0.019 and p=0.027, respectively). CONCLUSION: Changes in the choroid, CC and DCP are more frequently found than in the SCP on wide-field OCTA in intermediate uveitis. While wide-field OCTA is a reliable tool to detect capillary non-perfusion in intermediate uveitis, it was not helpful in determining disease activity. TRIAL REGISTRATION NUMBER: NCT02811536.
Assuntos
Vasculite Retiniana/patologia , Vasos Retinianos/patologia , Uveíte Intermediária/patologia , Adulto , Idoso , Corioide/irrigação sanguínea , Estudos Transversais , Feminino , Angiofluoresceinografia/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Retina/patologia , Tomografia de Coerência Óptica/métodosAssuntos
Aneurisma/diagnóstico , Complicações na Gravidez/diagnóstico , Hemorragia Retiniana/diagnóstico , Vasculite Retiniana/diagnóstico , Retinite/diagnóstico , Adulto , Aneurisma/complicações , Aneurisma/patologia , Aneurisma/cirurgia , Progressão da Doença , Feminino , Angiofluoresceinografia , Fundo de Olho , Humanos , Fotocoagulação , Gravidez , Complicações na Gravidez/patologia , Complicações na Gravidez/cirurgia , Hemorragia Retiniana/complicações , Hemorragia Retiniana/patologia , Hemorragia Retiniana/cirurgia , Vasculite Retiniana/complicações , Vasculite Retiniana/patologia , Vasculite Retiniana/cirurgia , Retinite/complicações , Retinite/patologia , Retinite/cirurgia , SíndromeRESUMO
Thousands of human disease-associated single nucleotide polymorphisms (SNPs) lie in the non-coding genome, but only a handful have been demonstrated to affect gene expression and human biology. We computationally identified risk-associated SNPs in deeply conserved non-exonic elements (CNEs) potentially contributing to 45 human diseases. We further demonstrated that human CNE1/rs17421627 associated with retinal vasculature defects showed transcriptional activity in the zebrafish retina, while introducing the risk-associated allele completely abolished CNE1 enhancer activity. Furthermore, deletion of CNE1 led to retinal vasculature defects and to a specific downregulation of microRNA-9, rather than MEF2C as predicted by the original genome-wide association studies. Consistent with these results, miR-9 depletion affects retinal vasculature formation, demonstrating MIR-9-2 as a critical gene underpinning the associated trait. Importantly, we validated that other CNEs act as transcriptional enhancers that can be disrupted by conserved non-coding SNPs. This study uncovers disease-associated non-coding mutations that are deeply conserved, providing a path for in vivo testing to reveal their cis-regulated genes and biological roles.
Assuntos
Elementos Facilitadores Genéticos/genética , MicroRNAs/genética , Vasculite Retiniana/genética , Alelos , Animais , Sequência Conservada/genética , Modelos Animais de Doenças , Regulação da Expressão Gênica/genética , Estudo de Associação Genômica Ampla , Humanos , Fatores de Transcrição MEF2/genética , Mutação , Polimorfismo de Nucleotídeo Único/genética , Retina/metabolismo , Retina/patologia , Vasculite Retiniana/patologia , Peixe-Zebra/genéticaAssuntos
Edema Macular/complicações , Vasculite Retiniana/complicações , Adolescente , Antituberculosos/uso terapêutico , Doxiciclina/uso terapêutico , Feminino , Angiofluoresceinografia , Humanos , Índia , Edema Macular/diagnóstico por imagem , Edema Macular/tratamento farmacológico , Edema Macular/patologia , Vasculite Retiniana/diagnóstico por imagem , Vasculite Retiniana/tratamento farmacológico , Vasculite Retiniana/patologia , Tomografia de Coerência ÓpticaRESUMO
PURPOSE: Eales' disease (ED), an enigmatic inflammatory disease, affects peripheral retinal veins and thereby vision in males. This study was aimed at identifying and deciphering the role of a novel 88-kDa protein reported in the serum and vitreous of patients with ED. EXPERIMENTAL DESIGN: The purified 88-kDa protein was identified by UPLC coupled ESI-QTOF-MS. The identified proteins were quantified in the serum from 20 ED patients and controls (age and sex matched), respectively by ELISA. The interaction of these proteins was studied using co-immunoprecipitation, western blot, and MS analyses. N-glycosylation of protein was observed by MS and lectin blot. RESULTS: The 88-kDa protein was identified to be a complex of haptoglobin, complement C3, and galectin-1. ELISA results showed a 1.5-fold increase in levels of haptoglobin (p = 0.008), with level of complement C3 unaltered and 1.2-fold decreased serum galectin-1 levels (p = 0.003) in ED patients compared to controls. Co-immunoprecipitation illustrated the interaction between haptoglobin and complement C3. Reduced sialylation and increased ß-1, 6-N-acetyl-glucosamine branched N-glycans were observed in haptoglobin of ED patients. CONCLUSION: The 88-kDa protein, a complex of haptoglobin, complement C3, and galectin-1, may play a potential role in ED pathogenesis while levels galectin-1 and haptoglobin may serve as potential biomarker of ED.
Assuntos
Complemento C3/análise , Galectina 1/sangue , Haptoglobinas/análise , Neovascularização Patológica/patologia , Vasculite Retiniana/patologia , Espectrometria de Massas por Ionização por Electrospray , Adolescente , Adulto , Estudos de Casos e Controles , Cromatografia Líquida de Alta Pressão , Complemento C3/metabolismo , Bases de Dados de Proteínas , Ensaio de Imunoadsorção Enzimática , Galectina 1/metabolismo , Haptoglobinas/metabolismo , Humanos , Imunoprecipitação , Pessoa de Meia-Idade , Neovascularização Patológica/metabolismo , Vasculite Retiniana/metabolismo , Adulto JovemRESUMO
PURPOSE: Describe the clinical features and outcomes of patients with peripheral retinal vasculitis (RV) and describe clinical characteristics of presumed tubercular RV in a nonendemic setting. METHODS: Retrospective cohort study of 110 consecutive patients with peripheral RV at a tertiary referral eye care center in the United Kingdom. Retinal vasculitis was defined as RV with vitritis associated with peripheral retinal ischemia. Patients who also had positive Quantiferon Gold in Tube test, positive tuberculin skin test, and/or other evidence of systemic tuberculosis such as biopsy were labeled with presumed tubercular RV. Treatment success was defined as resolution of inflammation, and successful tapering of oral corticosteroids to less than 10 mg/day or topical steroids to less than twice a day. RESULTS: Mean age of the study population was 42.69 ± 14.95 years. Patients were predominantly Asian (49.1%) and Male (67.0%). A total of 73.2% had bilateral involvement. Sixty-nine (62.72%) patients had presumed tubercular RV. A total of 52.8% patients received antitubercular therapy, 65.5% received oral corticosteroids, and 17.3% required steroid-sparing oral immunosuppressive agents. A total of 85.19% of patients with presumed tubercular RV achieved treatment success with concurrent antitubercular therapy as against 75.61% of patients with nontubercular RV. CONCLUSION: This is the largest study of the epidemiology, clinical features, and outcomes of both peripheral RV and presumed tubercular RV to date. Presumed tubercular RV commonly seems to affect young males of Asian descent and had vitreous hemorrhage as common clinical findings and also demonstrated a good treatment outcome with antitubercular therapy.
Assuntos
Corticosteroides/uso terapêutico , Imunossupressores/uso terapêutico , Vasculite Retiniana , Esteroides/uso terapêutico , Tuberculose Ocular/complicações , Adulto , Antituberculosos/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Vasculite Retiniana/tratamento farmacológico , Vasculite Retiniana/epidemiologia , Vasculite Retiniana/etiologia , Vasculite Retiniana/patologia , Estudos Retrospectivos , Tuberculose Ocular/tratamento farmacológico , Reino Unido/epidemiologia , Acuidade Visual , Adulto JovemRESUMO
ABSTRACT Lichen planus (LP) is an autoimmune, inflammatory disease of unknown etiology that commonly affects the skin and mucous membranes. Retinal vasculitis is a group of vision-threatening disorders, in which autoimmunity is thought to play a role in pathogenesis. We present the case of a patient who was diagnosed with retinal vasculitis and who was followed up for mucosal LP. LP has not been reported as a cause of retinal vasculitis in the literature. We believe that the retinal vasculitis in this case was related to LP because cellular immunity plays a role in the pathogenesis of both entities.
RESUMO Líquen plano (LP) é uma doença auto-imune, inflamatória de etiologia desconhecida que normalmente afeta a pele e membranas mucosas. Vasculite retiniana engloba um grupo de doenças que ameaçam a visão em que a autoimunidade parece desempenhar um papel na sua patogênese. Apresentamos um caso que foi diagnosticado com vasculite da retina e que tinha sido acompanhado com LP de mucosa. LP não consta entre as causas de vasculite retiniana na literatura. Acreditamos que a vasculite da retina no nosso caso, foi relacionado com o LP considerando que a imunidade celular desempenha um papel na patogênese de ambas as entidades.
Assuntos
Humanos , Masculino , Adulto , Vasculite Retiniana/diagnóstico , Líquen Plano/diagnóstico , Doenças Autoimunes/complicações , Angiofluoresceinografia , Vasculite Retiniana/etiologia , Vasculite Retiniana/patologia , Líquen Plano/complicaçõesRESUMO
INTRODUCTION: Rickettsial infections are re-emerging in the Indian subcontinent, especially among children. Understanding geographical and clinical epidemiology will facilitate early diagnosis and management. METHODOLOGY: Children aged <18yrs hospitalized with clinically-diagnosed rickettsial fever were reviewed retrospectively. Frequency distributions and odds ratios were calculated from tabulated data. RESULTS: Among 262 children hospitalized between January 2008-December 2012, median age was five years, and 61% were male children. Hospitalized cases increased steadily every year, with the highest burden (74%) occurring between September and January each year. Mean duration of fever was 11.5 days. Rash was present in 54.2% (142/262) of children, with 37.0% involving palms and soles. Prevalence of malnutrition was high (45% of children were underweight and 28% had stunting). Retinal vasculitis was seen in 13.7% (36/262), and the risk appeared higher in females. Severe complications were seen in 29% (purpura fulminans, 7.6%; meningitis and meningoencephalitis, 28%; septic shock, 1.9%; acute respiratory distress syndrome, 1.1%). Complications were more likely to occur in anemic children. Positive Weil-Felix test results (titers ≥1:160) were seen in 70% of cases. Elevated OX-K titers suggestive of scrub typhus were seen in 80% (147/184). Patients were treated with chloramphenicol (32%) or doxycycline (68%). Overall mortality among hospitalised children was 1.9%. CONCLUSIONS: This five-year analysis from southern India shows a high burden and increasing trend of rickettsial infections among children. The occurrence of retinal vasculitis and a high rate of severe complications draw attention to the need for early diagnosis and management of these infections.
Assuntos
Infecções por Rickettsiaceae/epidemiologia , Infecções por Rickettsiaceae/patologia , Adolescente , Criança , Pré-Escolar , Demografia , Feminino , Humanos , Índia/epidemiologia , Lactente , Masculino , Prevalência , Vasculite Retiniana/epidemiologia , Vasculite Retiniana/patologia , Estudos Retrospectivos , Infecções por Rickettsiaceae/complicaçõesAssuntos
Pan-Uveíte/patologia , Vasculite Retiniana/patologia , Tuberculose Ocular/patologia , Adulto , Diagnóstico Diferencial , Hemianopsia/microbiologia , Hemianopsia/patologia , Humanos , Isquemia/microbiologia , Isquemia/patologia , Masculino , Pan-Uveíte/microbiologia , Vasculite Retiniana/microbiologia , Tuberculose Ocular/complicaçõesAssuntos
Neovascularização Patológica/complicações , Vasculite Retiniana/complicações , Tuberculose Ocular/complicações , Adulto , Antituberculosos/uso terapêutico , Humanos , Masculino , Neovascularização Patológica/tratamento farmacológico , Neovascularização Patológica/patologia , Neovascularização Patológica/cirurgia , Vasculite Retiniana/tratamento farmacológico , Vasculite Retiniana/patologia , Vasculite Retiniana/cirurgia , Tuberculose Ocular/tratamento farmacológico , Tuberculose Ocular/patologia , Tuberculose Ocular/cirurgiaRESUMO
Lichen planus (LP) is an autoimmune, inflammatory disease of unknown etiology that commonly affects the skin and mucous membranes. Retinal vasculitis is a group of vision-threatening disorders, in which autoimmunity is thought to play a role in pathogenesis. We present the case of a patient who was diagnosed with retinal vasculitis and who was followed up for mucosal LP. LP has not been reported as a cause of retinal vasculitis in the literature. We believe that the retinal vasculitis in this case was related to LP because cellular immunity plays a role in the pathogenesis of both entities.
Assuntos
Líquen Plano/diagnóstico , Vasculite Retiniana/diagnóstico , Adulto , Doenças Autoimunes/complicações , Angiofluoresceinografia , Humanos , Líquen Plano/complicações , Masculino , Vasculite Retiniana/etiologia , Vasculite Retiniana/patologiaRESUMO
Frosted branch angiitis is a special form of retinal vasculitis, which has only rarely been reported in the literature. Although the majority of cases of frosted branch angiitis are idiopathic, a systematic clinical work-up should be done in order to exclude other causes, such as infectious and autoimmune diseases. The aim of this study was to correlate the clinical findings with the images obtained during follow-up by spectral domain optical coherence tomography (OCT).