Clinical characteristics and treatment of pars planitis: an adalimumab experience.

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.

Implications of pars planitis-associated cystoid macular edema on visual outcome and management in children.

PURPOSE: Pars planitis is a commonly observed type of pediatric uveitis. The aim of this study was to evaluate the implications of pars planitis-associated cystoid macular edema (CME) on visual outcome and treatment modalities. METHODS: A retrospective review of medical records in a single center with academic practice. RESULTS: Included were 33 children (mean age 8 years, 58 eyes). Eighteen eyes developed CME (31%): in 67% of them, CME was diagnosed at presentation and in 33%, it developed at a mean of 57 months after presentation. Anterior and posterior segment complications were more prevalent in eyes with CME. Papillitis was significantly associated with the development of CME (OR 12.4, 95% CI 2.3 to 65.6, p = 0.003). Patients with CME were 1.7 times more likely to be treated with systemic therapy. By the last follow-up, 50% of patients who never developed CME were without systemic therapy compared with 13% of patients who developed CME (p = 0.034). LogMAR visual acuity improvement between presentation and month 36 was 0.41 for eyes with CME compared with 0.14 for eyes that never developed CME (p = 0.009). CONCLUSION: Pars planitis-associated CME entailed higher prevalence of ocular complications, more frequent use of immunomodulatory therapy, and a lower rate of remission.

Clinical Presentation, Management, and Long-Term Outcome of Pars Planitis, Panuveitis, and Vogt-Koyanagi-Harada Disease in Children and Adolescents.

OBJECTIVE: Chronic uveitis is a common manifestation of pediatric rheumatologic conditions and may result in irreversible blindness and long-term disability. While chronic anterior uveitis is the most commonly encountered ocular manifestation of rheumatic disease, little is known about the clinical presentation, management, and long-term outcome of more complex eye conditions such as pars planitis (PP), panuveitis (PU), and Vogt-Koyanagi-Harada disease (VKH). The present study was undertaken to comprehensively assess the long-term safety and efficacy of disease-modifying antirheumatic drugs (DMARDs) and biologics for the treatment of pediatric and adolescent patients with PP, PU, and VKH. METHODS: We retrospectively reviewed a cohort of 75 children and adolescents with idiopathic PP (n = 50), PU (n = 12), and VKH (n = 14) followed by the Pediatric Rheumatology Core at Children's Hospital Los Angeles and evaluated referral patterns, clinical presentation, treatment response, and long-term clinical outcome. RESULTS: Patients were followed for an average of 52 months. Their mean age at disease onset was 10 years. Bilateral eye involvement was seen in 87% of the patients. At first presentation to an ophthalmologist, glaucoma was noted in 21% of patients and vision loss (<20/40) in 87% of patients, while legal blindness (≤20/200 in the better-seeing eye) was diagnosed in 18 of 75 (24%) of patients (PP 22%, PU 36%, and VKH 21%). The average referral time to a pediatric rheumatologist was 13 months (range 1-96 months). Topical steroids were used in all patients, but 98% of patients required additional DMARDs, and 73% required therapy with biologics. After a mean of 52 months, 35% of patients across all disease groups had significant vision loss or were blind, and only 28% were in clinical remission without medications. The worst outcome was observed in children with PU. Regression analysis, young age at onset, delayed referral to a pediatric rheumatologist, and chronic disease were strong predictors for the risk of long-term blindness. CONCLUSION: PP, PU, and VKH involve a high risk of permanent vision loss and should be managed by a skilled rheumatologist as early and as aggressively as possible.

Child With Red Eye and Blurry Vision.

Point-of-care ocular ultrasound can provide the clinician with more information about potential intraorbital and extraocular pathology, especially in cases when direct visualization of the eye is limited. This case report describes the findings in a pediatric patient who presented with a 1-month history of eye injection and worsening blurry vision. After point-of-care ultrasound demonstrated abnormal debris in the posterior vitreous cavity, subsequent evaluation revealed a diagnosis of pars planitis.

Pars Planitis en la infancia / Pars Planitis in childhood

Objetivo: Evaluar la respuesta al tratamiento con corticoides e inmunomoduladores en niños con pars planitis. Materiales y método: Se realiza un estudio retrospectivo y observacional de nueve historias clínicas de pacientes con diagnóstico de pars planitis atendidos en el servicio de oftalmología del Hospital de Pediatría Juan P. Garrahan desde el año 2010. Resultados: De los nueve pacientes 6 eran niños y 3 niñas. El motivo de consulta principal fue disminución de la visión. La mayoría de los pacientes fueron derivados por otros oftalmólogos para tratamiento. El 100% sufrió afectación ocular bilateral. Las complicaciones fueron: catarata, queratopatía en banda, hipertensión ocular y edema macular cistoide. El 100% de los pacientes recibió tratamiento con corticoides por vía oral e inmunomoduladores. A algunos se les efectuó inyecciones de triamcinolona subtenoniana y criocoagulación. Conclusiones: con el tratamiento con prednisona e inmunomoduladores se logró mejoría en la agudeza visual en 15 ojos y 3 ojos mantuvieron igual agudeza visual. La pars planitis es una patología crónica con un pronóstico visual reservado que requiere de un seguimiento estrecho y tratamiento prolongado por parte de un equipo interdisciplinario (AU)

Objective: To assess response to steroid and immunomodulating treatment in children with pars planitis. Material and methods: A retrospective observational study was conducted reviewing nine clinical charts of children with a diagnosis of pars planitis seen at the Department of Ophthalmology of the Pediatric Hospital Juan P. Garrahan since 2010. Results: Of nine patients, six were boys and three were girls. Main complaint was loss of vision. The majority of patients were referred to our hospital by other ophthalmologists for treatment. All children had bilateral eye involvement. Complications observed were: cataracts, band keratopathy, ocular hypertension, and cystoid macular edema. All patients received oral steroids and immunomodulating treatment. In some subtenonian injection of triamcinolone and cryocoagulation was performed. Conclusions: Prednisone and immunomodulating treatment resulted in an improvement of visual acuity in 15 eyes and three eyes visual acuity remained unchanged. Pars planitis is a chronic disease with an uncertain visual prognosis that requires close follow-up and prolonged treatment by an interdisciplinary team (AU)

Treatment with immunosuppressive therapy in patients with pars planitis: experience of a reference centre in Mexico.

AIM: To evaluate the clinical course of the patients with pars planitis that received immunosuppressive drugs. METHODS: We retrospectively analysed the data of 10 years from 374 patients with pars planitis in a large reference centre in Mexico City and included 49 patients (92 eyes). RESULTS: Median age at presentation was 8 years. 35 patients (71.4%) were male and 43 patients (87.7%) had bilateral disease. Diverse immunosuppressive medications were used, mainly methotrexate (69.4%) and azathioprine (63.3%) with 18 patients requiring more than one drug. The main indications for starting immunosuppressive therapy were lack of response to initial treatment and advance disease at presentation. The results showed good response with steroid reduction (69.3% of patients), visual acuity improvement (51% of patients) and inflammatory disease reduction (59.1% of patients). In 25 patients (51%), steroids were started previous to immunosuppressors and in 24 (49%) at the same time without significant difference in clinical improvement (p=0.210) or visual outcome (p=0.498). Thirteen patients (26.5%) presented mild adverse effects. The median of the final visual acuity was 20/40. The median follow-up time was 44 months (range 13-115 months). CONCLUSIONS: Immunosuppressive therapy allows an adequate control of inflammatory disease in pars planitis, with clinical and visual improvement and steroid dose reduction.

Rheumatic inflammatory eye diseases of childhood.

Chronic inflammatory eye diseases are a common manifestation of pediatric rheumatologic diseases, potentially leading to lifelong vision impairment and disability. The mechanisms leading to the breach of the blood eye/brain barrier and the subsequent immune attack against a variety of intraocular mostly unidentified antigens remains poorly understood. Pediatric rheumatologists need to be familiar with the various inflammatory eye diseases because they are often responsible for selecting and supervising treatment in close collaboration with the ophthalmologist. This article provides an update of recent developments in the pathogenesis and treatment of the most relevant ocular diseases encountered in rheumatologic practice.

Different ophthalmologic manifestations of sarcoidosis.

PURPOSE OF REVIEW: Sarcoidosis can manifest with different ocular findings. Three different cases have been presented, each of which showed different ocular problems. The literature has also been reviewed as to find out other eye signs and treatment strategies of the disease. The diagnosis may be difficult and the treatment may include combination of different immunosuppressors. RECENT FINDINGS: Recent findings include a genetic basis, and certain human leukocyte antigens may affect the course of the disease. Sarcoidosis can influence the eye and the optic nerves in the beginning, and biopsy of the involved tissue may be necessary for the diagnosis. Laboratory investigation may be unyielding. Once the diagnosis is made, steroids are generally started. Other than the classical corticosteroid treatment, other immunosuppressive agents show promise in the atypical cases. SUMMARY: Our cases show different manifestations of the disease like bilateral optic neuropathy, Horner's syndrome, pars planitis, and anterior and posterior uveitis. Patients recovered with steroid treatment, but especially in young patients other agents like methotrexate were needed because of the sideeffects of steroids.

Effects of twice-daily topical difluprednate 0.05% emulsion in a child with pars planitis.

PURPOSE: To report the effects of twice-daily difluprednate in a child with pars planitis (PP). DESIGN/METHODS: Case report. RESULTS: PP was controlled with topical difluprednate for 1 year. Then an atypical pattern of steroid response--delayed, relatively sudden onset of recalcitrant ocular hypertension (OHT)--and posterior subcapsular cataract (PSC) formation necessitated alternative treatment. CONCLUSION: Although not a standard treatment, in select cases of PP topical difluprednate therapy could be a useful short-term treatment option while alternative treatments are considered or immunosuppressive agents build to therapeutic levels. Ophthalmologists must be aware of the potential for delayed onset of serious complications when using difluprednate.

[Pars plana vitrectomy as anti-inflammatory therapy for intermediate uveitis in children]. / La vitrectomía como terapia antinflamatoria de la uveítis intermedia en niños.

PURPOSE: Intermediate uveitis represents between 2 and 26% of uveitis in children. The spectrum of the disease is highly variable, ranging between mild cases that resolve spontaneously and chronic, severe forms that develop multiple episodes and complications. The purpose of this study is to evaluate the efficacy of vitrectomy to control inflammation in children with recurrent intermediate uveitis. METHODS: Retrospective evaluation of patients with at least six months of follow-up. All patients under 16 who had undergone vitrectomy for intermediate uveitis were included. Vitrectomy was performed after at least two episodes of intermediate uveitis in children that had had no previous prophylactic systemic immunosuppressant treatment. Data recorded were visual acuity (VA), recurrences and surgical complications. RESULTS: Seven eyes of five children with intermediate uveitis who underwent vitrectomy were included. After a mean follow-up of 34 months, VA improved in all eyes after surgery. Four eyes developed mild subcapsular posterior cataracts. Post-surgical recurrences were anterior and responded to topical treatment, except for an episode of intermediate uveitis that required a periocular injection of triamcinolone. Only one patient is being treated with systemic immunosuppressants, due to the presence of repeated episodes of uveitis in the non-vitrectomised eye and since his parents were unwilling to have him undergo new surgery. CONCLUSIONS: Vitrectomy with inferior cryotheraphy controls inflammation in intermediate uveitis in children with good mid-term results avoiding the secondary side-effects of systemic immunosuppressants.

Management of persistent glaucoma secondary to depot methylprednisolone.

This article describes two patients with recalcitrant ocular hypertension persisting 6 and 4 months, respectively, after periocular injection of methylprednisolone acetate despite maximum medical therapy. White sub-Tenon's plaques of residual methylprednisolone acetate were excised surgically and analyzed for steroid activity using spectrophotometry. In both cases, intraocular pressure was normalized promptly after surgical removal of visible steroid. In both instances, biochemical analysis of the excised samples revealed residual steroid. Surgical removal of a residual methylprednisolone depot is an effective management choice in patients developing persistent intraocular pressure elevation after periocular injection.

Valsalva retinopathy: diagnostic challenges in a patient with pars-planitis.

PURPOSE: To describe a case of Valsalva retinopathy in a patient with pars-planitis and to discuss its management. METHODS: We present a case of a 19-year-old male with a six-month history of bilateral intermediate uveitis, referred for evaluation of recent-onset spontaneous bilateral vitreous hemorrhages. Clinical evaluation and fluorescein angiography were negative for the presence of neovascularization. On closer questioning the patient revealed that he had his most recent symptoms after heavy weight lifting. RESULTS: The patient was instructed to avoid heavy weight lifting. Over the next two months, vitreal hemorrhages had resolved. However, despite the treatment with systemic prednisone, the patient's intermediate uveitis was still active, and cystoid macular edema had developed. Therefore, we proceeded to systemic immunomodulatory therapy, which controlled the inflammation and preserved vision in each eye. CONCLUSIONS: Although a very rare cause, Valsalva maneuver should be included in the differential diagnosis of bilateral vitreous hemorrhages. Ophthalmologists should advise patients with known eye problems that can predispose to vitreal hemorrhages to refrain from it.

Pars planitis in the Mexican Mestizo population: ocular findings, treatment, and visual outcome.

PURPOSE: To describe the clinical manifestations of classic pars planitis (CPP) in Mexican patients. We report here the most frequent complications, medical and surgical treatment, and visual prognosis. MATERIAL AND METHODS: A retrospective, descriptive case series examined the clinical features, complications, and treatment (medical and surgical) of CPP patients seen at the Inflammatory Eye Disease Clinic from January 1990 to September 1999. RESULTS: One hundred and sixty patients met inclusion criteria for the study. Mean age at presentation was 10 years and males were more frequently affected. Both eyes were affected in 84.4% of the cases. The most frequent complaint was decreased visual acuity. Initial visual acuity (VA) ranged from no light perception to 20/20 (mean 20/50), and mean final VA was 20/30. The most frequent clinical manifestations were vitritis (99.7%), snowballs (99.3%), retinal vasculitis (89.2%), and snowbanks (63.1%). The most common complications were cystoid macular edema (63.4%) and cataract (47.5%). Periocular corticosteroids were used in 97.5% of cases, systemic corticosteroids in 68.1%, and other immunosuppressive drugs in 21.3%. CONCLUSIONS: CPP in the Mexican population is more frequent in males and usually presents in patients less than 14 years of age. It is typically bilateral, and the most common symptom is decreased visual acuity. The most important clinical findings are located in the vitreous and retina. Cataract and cystoid macular edema are the most frequent complications. Treatment comprises periocular and systemic corticosteroids or other immunosuppressive drugs.

Inflammatory bowel disease (Crohn's disease) in a Spanish patient with pars plana exudates: report of a new case and review of the literature.

Several ocular manifestations have been found in Crohn's disease patients, most often affecting the anterior segment. This paper presents the case of a young woman with pars plana exudates in whom Crohn's disease was later diagnosed. To the authors' knowledge, this is only the second report of Crohn's disease and concomitant pars plana exudates.

Lower eyelid herniation of orbital fat may complicate periocular corticosteroid injection.

PURPOSE: To report a case of lower eyelid herniation of orbital fat occurring after periocular corticosteroid injection. DESIGN: Interventional case report. METHODS: A 44-year-old man with asymmetrical pars planitis complicated by right cystoid macular edema was treated with multiple right orbital floor injections of triamcinolone through the lower eyelid. RESULTS: Right lower eyelid orbital fat herniation occurred during the course of the treatment. CONCLUSION: A herniation of orbital fat may complicate the injection of corticosteroid through the lower eyelid.

Circulating antibodies to inducible heat shock protein 70 in patients with uveitis.

Heat shock proteins with molecular weight 70 kDa (hsp70) are highly conserved immunogenic intracellular molecules. There are two main subtypes: one is expressed constitutively (hsc70), while the other is induced under stressful conditions (ihsp70). Using an ELISA directed against recombinant human ihsp70, antibody titers were determined in patients with defined ocular inflammatory conditions (Behçet's disease, Vogt-Koyanagi-Harada (VKH), pars planitis, and sarcoidosis) as well as in a group of age-matched normal volunteers. In comparison to healthy controls (n = 14, absorbance 0.269), levels were significantly elevated in Behçet's disease (n = 18; 0.412), sarcoidosis (n = 15; 0.432), and pars planitis (n = 13; 0.346), but not in VKH (n = 10; 0.263). A correlation was also noted for treatment versus no treatment in pars planitis (p = 0.028), but not in other inflammatory conditions. There was no correlation with the level of intraocular disease activity as defined by vitreous haze and vision drop. Since pars planitis is a purely ocular condition, circulating levels of ihsp antibodies likely reflect the extent of disease involvement within the eye.