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Ocular Immunology and Inflammation Jul 2023This review aims to provide an update on the clinical presentation, etiologies, complications, and treatment options in intermediate uveitis (IU). (Review)
Review
PURPOSE
This review aims to provide an update on the clinical presentation, etiologies, complications, and treatment options in intermediate uveitis (IU).
METHODS
Narrative literature review.
RESULTS
IU affects all age groups with no clear gender predominance and has varied etiologies including systemic illnesses and infectious diseases, or pars planitis. In some instances, IU may be the sole presentation of an underlying associated condition or disease. Management of IU and its complications include administration of corticosteroids, antimetabolites, T-cell inhibitors, and/or biologics, along with surgical interventions, with varying degrees of effectiveness across literature. In particular, increasing evidence of the safety and efficacy of immunomodulatory agents and biologics has seen greater adoption of these therapies in clinical practice.
CONCLUSIONS
IU is an anatomical description of uveitis, involving intraocular inflammation of the vitreous, peripheral retinal vasculature, and pars plana. Various treatment options for intermediate uveitis are currently used in practice.
Topics: Humans; Uveitis, Intermediate; Pars Planitis; Uveitis; Vision Disorders; T-Lymphocytes
PubMed: 35759636
DOI: 10.1080/09273948.2022.2070503 -
Survey of Ophthalmology 2022Pediatric uveitis accounts for 5-10% of all uveitis. Uveitis in children differs from adult uveitis in that it is commonly asymptomatic and can become chronic and cause... (Review)
Review
Pediatric uveitis accounts for 5-10% of all uveitis. Uveitis in children differs from adult uveitis in that it is commonly asymptomatic and can become chronic and cause damage to ocular structures. The diagnosis might be delayed for multiple reasons, including the preverbal age and difficulties in examining young children. Pediatric uveitis may be infectious or noninfectious in etiology. The etiology of noninfectious uveitis is presumed to be autoimmune or autoinflammatory. The most common causes of uveitis in this age group are idiopathic and juvenile idiopathic arthritis-associated uveitis. The stepladder approach for the treatment of pediatric uveitis is based on expert opinion and algorithms proposed by multidisciplinary panels. Uveitis morbidities in pediatric patients include cataract, glaucoma, and amblyopia. Pediatric patients with uveitis should be frequently examined until remission is achieved. Once in remission, the interval between follow-up visits can be extended; however, it is recommended that even after remission the child should be seen every 8-12 weeks depending on the history of uveitis and the medications used. Close follow up is also necessary as uveitis can flare up during immunomodulatory therapy. It is crucial to measure the impact of uveitis, its treatment, and its complications on the child and the child's family. Visual acuity can be considered as an acceptable criterion for assessing visual function. Additionally, the number of cells in the anterior chamber can be a measure of disease activity. We review different aspects of pediatric uveitis. We discuss the mechanisms of noninfectious uveitis, including autoimmune and autoinflammatory etiologies, and the risks of developing uveitis in children with systemic rheumatologic diseases. We address the risk factors for developing morbidities, the Standardization of Uveitis Nomenclature (SUN) criteria for timing and anatomical classifications, and describe a stepladder approach in the treatment of pediatric uveitis based on expert opinion and algorithms proposed by multi-disciplinary panels. In this review article, We describe the most common entities for each type of anatomical classification and complications of uveitis for the pediatric population. Additionally, we address monitoring of children with uveitis and evaluation of Quality of Life.
Topics: Adult; Cataract; Child; Child, Preschool; Humans; Quality of Life; Retrospective Studies; Uveitis; Visual Acuity
PubMed: 34181974
DOI: 10.1016/j.survophthal.2021.06.006 -
Ocular Immunology and Inflammation Dec 2023To provide an overview of pediatric pars planitis. (Review)
Review
PURPOSE
To provide an overview of pediatric pars planitis.
METHODS
Narrative literature review.
RESULTS
Pars planitis refers to the idiopathic subset of intermediate uveitis in which there is vitritis along with snowball or snowbank formation occurring in the absence of an associated infection or systemic disease. It is thought to be a T-cell mediated disease with a genetic predisposition. Pars planitis accounts for 5-26.7% of pediatric uveitis cases. Presentation is commonly bilateral but asymmetric, often with insidious onset of floaters and blurred vision. Although pars planitis is known to be a benign form of uveitis in most cases, severe complications secondary to chronic inflammation may arise, with cystoid macular edema being the most common cause of visual morbidity. Mild vitritis in the absence of symptoms, vision loss, or macular edema may be observed. Patients with severe vitritis and/or associated vision-threatening complications require prompt aggressive treatment. A stepladder approach including corticosteroids, immunosuppressive agents, anti‑tumor necrosis factor‑alpha and pars plana vitrectomy and/or laser photocoagulation is the most commonly used method for treatment of pars planitis.
CONCLUSION
Timely diagnosis and adequate treatment of pediatric pars planitis and associated complications are crucial in order to improve visual outcomes.
Topics: Humans; Child; Pars Planitis; Uveitis, Intermediate; Uveitis; Vitrectomy; Adrenal Cortex Hormones; Macular Edema; Endophthalmitis; Retrospective Studies
PubMed: 37976519
DOI: 10.1080/09273948.2023.2279683 -
Turkish Archives of Pediatrics Jul 2023Uveitis in childhood poses a distinct challenge, mainly because of the insidious onset and chronic course of intraocular inflammation in most cases, which may result in...
Uveitis in childhood poses a distinct challenge, mainly because of the insidious onset and chronic course of intraocular inflammation in most cases, which may result in permanent visual loss due to delayed diagnosis and treatment. Although anterior uveitis, frequently associated with juvenile idiopathic arthritis, is the most common form of ocular involvement, idiopathic intermediate uveitis (pars planitis) is also a common uveitic entity in childhood. Posterior or panuveitis of a variety of noninfectious or infectious etiologies may be seen as well. Pediatric uveitis needs to be closely monitored since serious ocular complications such as intraocular pressure elevation, cataract, and macular edema may rapidly develop due to inadequately controlled inflammation and/or the use of corticosteroids. Methotrexate is generally the first- line corticosteroid-sparing agent, and adalimumab is the first-line biologic in refractory cases of noninfectious uveitis. A multidisciplinary approach is essential to monitor systemic disease associations, treatment response, and adverse events in children with uveitis.
PubMed: 37357450
DOI: 10.5152/TurkArchPediatr.2023.23086 -
Journal of Ophthalmic & Vision Research 2015Pars planitis is an idiopathic chronic intermediate uveitis which predominantly affects children and adolescents, and accounts for 5-26.7% of pediatric uveitis. Although... (Review)
Review
Pars planitis is an idiopathic chronic intermediate uveitis which predominantly affects children and adolescents, and accounts for 5-26.7% of pediatric uveitis. Although an autoimmune process with a genetic predisposition has been suggested, its etiology still remains unknown. The most common presenting symptoms are floaters and blurred vision. Diffuse vitreous cells, haze, snowballs and snowbanks are typical findings of pars planitis. Peripheral retinal vasculitis, optic disc edema and anterior segment inflammation are other well-known findings. Although pars planitis is known to be a benign form of uveitis in most cases, it may become a potentially blinding disease due to complications including cataract, cystoid macular edema, vitreous opacities and optic disc edema. Cystoid macular edema is the most common cause of visual morbidity. Band keratopathy, epiretinal membrane formation, vitreous condensation, neovascularizations, vitreous hemorrhage, retinal detachment, cyclitic membranes, glaucoma and amblyopia may develop as a consequence of the chronic course of the disease. Exclusion of infectious and non-infectious causes which may present with intermediate uveitis is of utmost importance before starting treatment. Treatment of pars planitis has been a controversial issue. There is no consensus specifically for treatment of cases with minimal inflammation and relatively good visual acuity. However, current experience shows that pars planitis may cause severe inflammation and needs an aggressive treatment. A stepladder approach including corticosteroids, immunosupressive agents, anti-tumor necrosis factor-alpha and pars plana vitrectomy and/or laser photocoagulation is the most commonly used method for treatment of pars planitis. Adequate control of inflammation and prompt detection of associated complications are crucial in order to improve the overall prognosis of the disease.
PubMed: 27051493
DOI: 10.4103/2008-322X.176897 -
American Journal of Ophthalmology Aug 2021To determine classification criteria for pars planitis.
PURPOSE
To determine classification criteria for pars planitis.
DESIGN
Machine learning of cases with pars planitis and 4 other intermediate uveitides.
METHODS
Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set.
RESULTS
Five hundred eighty-nine cases of intermediate uveitides, including 226 cases of pars planitis, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for pars planitis included unilateral or bilateral intermediate uveitis with either 1) snowballs in the vitreous or 2) snowbanks on the pars plana. Key exclusions included: 1) multiple sclerosis, 2) sarcoidosis, and 3) syphilis. The misclassification rates for pars planitis were 0% in the training set and 1.7% in the validation set, respectively.
CONCLUSIONS
The criteria for pars planitis had a low misclassification rate and appeared to perform sufficiently well for use in clinical and translational research.
Topics: Adolescent; Adult; Child; Female; Humans; Machine Learning; Male; Pars Planitis; Visual Acuity; Young Adult
PubMed: 33845006
DOI: 10.1016/j.ajo.2021.03.045 -
Archivum Immunologiae Et Therapiae... Apr 2016Pars planitis is defined as an intermediate uveitis of unknown background of systemic disease with characteristic formations such as vitreous snowballs, snowbanks and... (Review)
Review
Pars planitis is defined as an intermediate uveitis of unknown background of systemic disease with characteristic formations such as vitreous snowballs, snowbanks and changes in peripheral retina. The incidence of pars planitis varies 2.4-15.4 % of the uveitis patients. The pathogenesis of the disease is to be determined in future. Clinical and histopathological findings suggest an autoimmune etiology, most likely as a reaction to endogenous antigen of unknown source, with T cells predominant in both vitreous and pars plana infiltrations. T cells subsets play an important role as a memory-effector peripheral cell. Snowbanks are formed as an effect of post inflammatory glial proliferation of fibrous astrocytes. There is also a genetic predisposition for pars planitis by human leukocyte antigen and several other genes. A coexistence of multiple sclerosis and optic neuritis has been described in numerous studies. Epiretinal membrane, cataract, cystoid macular edema, retinal detachment, retinal vasculitis, neovascularization, vitreous peripheral traction, peripheral hole formation, vitreous hemorrhage, disc edema are common complications observed in pars planitis. There is a need to expand the knowledge of the pathogenic and immunologic background of the pars planitis to create an accurate pharmacological treatment.
Topics: Animals; Autoimmunity; Eye; Genetic Predisposition to Disease; HLA Antigens; Humans; Immunologic Memory; Pars Planitis; T-Lymphocyte Subsets; T-Lymphocytes
PubMed: 26438050
DOI: 10.1007/s00005-015-0361-y -
Graefe's Archive For Clinical and... Feb 2022This study aims to investigate the clinical and demographic characteristics, treatment outcomes and complications of patients with pars planitis.
PURPOSE
This study aims to investigate the clinical and demographic characteristics, treatment outcomes and complications of patients with pars planitis.
METHODS
This retrospective study included patients diagnosed with pars planitis between 1998 and 2019 and followed for at least 6 months. Demographics, best-corrected visual acuity (BCVA), anterior segment and fundus examination findings, intraocular pressure (IOP) values at baseline and final examination, treatments used during the follow-up, surgeries and complications were noted from medical records of the patients. The percentage of patients given adalimumab (ADA), the reasons for treatment switch and response to ADA were investigated.
RESULTS
One hundred fifteen eyes of 59 patients were included in the study. Forty-seven percent of patients were female. The median age of the patients was 10 (4-44) years. The median follow-up time was 33 (6-252) months. The median BCVA at admission was 0.20 (0.00-2.00) logMAR. The most common complications were cystoid macular oedema, cataract, epiretinal membrane and inferior peripheral retinoschisis. Prophylactic laser photocoagulation for peripheral retinoschisis was the most common surgical intervention, followed by cataract surgery and pars plana vitrectomy. Approximately 80% of patients received immunosuppressive and corticosteroid therapy for initial treatment. ADA was initiated in 23 patients (38.9%) due to refractory uveitis and adverse effects to the corticosteroid and helped control intraocular inflammation and decrease the use of systemic steroids/immunosuppressives in 22 of 23 (95%) of patients who received ADA. The median BCVA at final examination increased to 0.00 (0.00-2.00) logMAR.
CONCLUSIONS
Pars planitis is a chronic, progressive and insidious disease with several ocular complications and requires early and aggressive treatment. ADA appeared to be effective especially in patients' refractory to conventional treatment.
Topics: Adalimumab; Adolescent; Adult; Child; Child, Preschool; Female; Humans; Male; Pars Planitis; Retrospective Studies; Visual Acuity; Vitrectomy; Young Adult
PubMed: 34568953
DOI: 10.1007/s00417-021-05398-4