RARE DISEASE
Non-infectious posterior uveitis
Non-infectious posterior uveitis
Non-infectious posterior uveitis
Synonyms: Non-infectious choroiditis
Synonyms: Non-infectious choroiditis
Synonyms: Non-infectious choroiditis
Drug discovery
8
drugs
With orphan designations
Overview
Non-infectious posterior uveitis (NIU-PS) is a sight-threatening intraocular inflammatory condition affecting the choroid, retina, or optic nerve, often linked to autoimmune disorders like Behçet’s disease, sarcoidosis, or Vogt-Koyanagi-Harada syndrome. It accounts for 10–15% of blindness in developed nations and requires prompt intervention to prevent complications such as macular edema, cataracts, and glaucoma. Current therapies prioritize inflammation control with corticosteroids and biologic agents, though long-term management challenges persist due to treatment side effects and disease recurrence [3][5][6][12].
Population
Prevalence of non-infectious uveitis is 121/100,000 adults in the U.S., with posterior segment involvement in ~45% of cases [2][15].
Higher prevalence in females (146/100,000 vs. 119/100,000 in males) and adults aged 65+ (190/100,000) [2][15].
Pediatric cases occur at 29/100,000, often linked to juvenile idiopathic arthritis [2][14].
Burden
Clinical: Vision loss occurs in 20–30% of cases; 70% develop complications (e.g., cataracts, glaucoma) [5][18].
Economic: Annual direct costs reach $52,500 for bilateral disease; indirect costs (work absenteeism) average $6,902/year [9][20].
Psychosocial: High rates of psychological distress (anxiety/depression) and reduced quality of life [5][18].
Therapies
First-line: Corticosteroids (oral, intravitreal, or implants like fluocinolone acetonide or dexamethasone) [6][20].
Steroid-sparing agents: Immunomodulators (methotrexate, mycophenolate) and biologics (adalimumab, infliximab; anti-TNF-α agents) [1][3][16].
Emerging options: Targeted biologics (tocilizumab, rituximab) and sustained-release implants to reduce systemic toxicity [6][12][18].
Categories: rare ophthalmic disorders
Research Papers
567 drug discovery papers about Non-infectious posterior uveitis, with 1 first-in-class and 4 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
567 drug discovery papers about Non-infectious posterior uveitis, with 1 first-in-class and 4 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
2026-08-08 | Adalimumab Therapy Reduces Treatment Burden in Multifocal Choroiditis/Punctate Inner Choroidopathy.
This study aims to demonstrate the efficacy of adalimumab (ADA) in reducing overall treatment burden in a cohort of patients with idiopathic multifocal choroiditis (MFC)/punctate inner choroidopathy (PIC). This is a retrospective case series of eyes classified as having idiopathic MFC/PIC using standard multi-modal imaging, including color fundus photography, fundus autofluorescence, and spectral-domain optical coherence tomography. LogMAR best-corrected visual acuity (BCVA), doses of prednisone and other immunomodulatory therapies (IMT), injections of corticosteroids or anti-vascular endothelial growth factor (anti-VEGF), and breakthrough flares were collected at baseline (initiation of ADA), 12 months from baseline, and last follow-up. Twenty-five patients (21 females, 41 eyes) were included. Mean follow-up time was 51.4 ± 35.2 months (range 9.2-126.6). Mean LogMAR BCVA was 0.35 ± 0.48 at baseline and 0.34 ± 0.55 at last follow-up (p > 0.5). Mean prednisone dosage decreased significantly from 15.76 ± 18.69 mg/day at baseline to 0.23 ± 1.1 mg/day at the last follow-up (p < 0.0001). Thirty eyes received steroid or anti-VEGF injections at baseline; this decreased to one at the last follow-up (p < 0.0001). Similarly, eight patients received other IMT at baseline, but only one continued to receive supplemental IMT at the last follow-up (p < 0.05) at a decreased dosage. Six patients experienced disease flare-up within the first 12 months of treatment, and three patients flared between 12 months and last follow-up. Adalimumab therapy demonstrated a significant steroid-sparing effect in addition to vision preservation and reduction in both anti-VEGF injections and IMT.
2026-07-17 | Demographic and socioeconomic associations with outcomes in pediatric uveitis: An IRIS® registry study
Objective To evaluate the associations of demographic and socioeconomic factors—including race and ethnicity, sex, insurance type, age, and geographic region—with visual outcomes, ocular complications, and the need for ocular surgery in children with non-infectious uveitis Design Retrospective cohort study using the American Academy of Ophthalmology IRIS® (Intelligent Research in Sight) Registry, a national ophthalmic disease registry Participants Pediatric patients with non-infectious uveitis Methods Patients with non-infectious uveitis diagnosed and treated at age ≤18 with ≥1 year follow-up with complete demographic information identified in the IRIS® Registry between January 1, 2013 and December 31, 2019. Insurance type was used as a proxy for socioeconomic status, and analyses accounting for the inclusion of both eyes of the same patient were performed using generalized estimating equations. Multivariable logistic or linear regression were performed to assess whether age, sex, race, region and insurance type were associated with higher risk of poor visual acuity outcomes, complications of uveitis and requirement for ocular surgery. Results Data from 3611 unique patients (5,723 eyes) were included. Compared to White children, Black children were more likely to have a final visual acuity of 20/200 or worse and develop glaucoma and cystoid macular edema (OR for White vs Black children for 20/200 or worse vision 0.59, 95% CI 0.42–0.82; P<0.05). Compared to children with private insurance, those with Medicaid were more likely to have a final visual acuity of 20/200 or worse, as well as suffer from amblyopia, posterior synechiae, and band keratopathy (OR for private vs Medicaid for 20/200 or worse vision 0.50, 95% CI 0.37–0.67; P<0.05 for all). Children with age ≤12 years were more likely to develop band keratopathy and amblyopia compared to those 13-18 years old (amblyopia OR 4.99, 95% CI 3.00–8.31; band keratopathy OR 2.62, 95% CI 1.89–3.63; P<0.001 for both). Conclusion Socioeconomic and demographic factors associated with poorer clinical outcomes in pediatric non-infectious uveitis include younger age at presentation, Black
2026-06-25 | Uveitis in spondyloarthritis: clinical patterns and differences between axial and peripheral forms.
The association between uveitis and spondyloarthritis is well established. However, its characterization across the different types of spondyloarthritis remains limited in the literature. Therefore, this study aimed to perform a comparative characterization of uveitis in the various types of spondyloarthritis. Retrospective observational study that included patients with non-infectious uveitis and spondyloarthritis from a multidisciplinary uveitis clinic. A descriptive analysis was performed across the different subtypes of spondyloarthritis, followed by a comparative analysis regrouping patients into axial and peripheral SpA categories. 163 patients with spondyloarthritis-associated uveitis were included, comprising radiographic axial spondyloarthritis (49%), non-radiographic axial spondyloarthritis (21%), spondyloarthritis associated with inflammatory bowel disease (13%), psoriatic arthritis (12%), and peripheral spondyloarthritis (4%). Anterior uveitis (97%), acute relapsing course (79%), and alternating laterality (47.4%) were predominant in the overall sample. When comparing axial versus peripheral forms, significant differences were observed: intermediate, posterior, and panuveitis locations occurred exclusively in the peripheral forms, which also showed higher frequencies of chronic courses (p < 0.001), bilateralism (p < 0.05), and greater use of systemic therapy (p = 0.05). Regarding this treatment to control uveitis, 28.2% of patients required immunomodulatory therapy (more sulfasalazine in axial forms, and methotrexate in peripheral) and 17.8% required biologic therapy. Biologic discontinuation was higher in peripheral forms (p = 0.059). Variables predicting greater need for systemic therapy included chronic course, bilateralism, higher number of annual episodes, younger age of onset, peripheral joint involvement, and presence of vitritis (all p < 0.05). Acute anterior uveitis constitutes the most frequent pattern in all spondyloarthritis types. However, peripheral forms exhibit a higher prevalence of non-anterior, chronic, and bilateral uveitis, and, additionally, appeared to require greater use of immunomodulatory therapy. Key Points • Understanding and distinguishing the ocular inflammatory processes associated with SpA may have important therapeutic and prognostic implications. • Although acute recurrent anterior uveitis was the most frequent patternacross all SpA subtypes, in the peripheral SpA group (pSpA, IBD-SpA, and PsA) a higher prevalence of intermediate, posterior, panuveitis, chronic course, and bilateral uveitis was observed. • The peripheral SpA group (pSpA, IBD-SpA, and PsA) appeared to require greater use of immunomodulatory drugs (csDMARDs or biologics) for control of the ocular condition, suggesting the need for closer monitoring and tailored therapeutic strategies.
2026-06-19 | Bromfenac 0.09% as adjunct to anti-VEGF for the treatment of macular edema secondary to noninfectious uveitis
Uveitis is a major cause of visual impairment and blindness and macular edema is the major risk factor. Uveitic macular edema (UME) mechanism is primarily through blood-retinal barrier breakdown driven by inflammatory mediators such as prostaglandins and vascular endothelial growth factor (VEGF). Corticosteroids are the main treatment but can cause significant side effects leading to the use of alternatives including topical non-steroidal anti-inflammatory drugs (NSAIDs) and intravitreal anti-VEGF agents. Bromfenac is an FDA-approved NSAID for postoperative inflammation but its efficacy in UME is not clear. We report a case of UME secondary to non-infectious uveitis managed with topical bromfenac in combination with intravitreal aflibercept. Case Presentation: A 13-year-old female with a history of chronic intermittent bilateral posterior uveitis presented for follow-up 6 months after stopping biologic treatment and 2 years of stable disease activity. Uncorrected visual acuity was 6/12 in both eyes (OU) and recurrent macular edema was found in the right eye (OD) with central foveal thickness (CFT) of 718 µm. The patient was started on bromfenac 0.09% twice daily and received a single intravitreal anti-VEGF injection. At one-week follow-up, the CFT decreased to 419 µm. At four-week follow-up, the CFT decreased to 318 µm OD and there was significant improvement of the macular edema.Conclusion: Bromfenac 0.09% as adjunctive to anti-VEGF injection may be an effective treatment for macular edema secondary to non-infectious uveitis.
2026-04-29 | Demographic and clinical pattern of non-infectious uveitis in the Tripoli Children's Hospital
Uveitis is relatively rare in the pediatric population, but it leads to considerable ocular morbidity. This study aims to describe the clinical, etiological, and treatment features of noninfectious uveitis in Libyan children in a pediatric rheumatology clinic. A retrospective analysis of medical records of pediatric patients who were diagnosed with noninfectious uveitis from January 2000 to December 2021 at the pediatric rheumatology clinic at Tripoli Children's Hospital, Tripoli, Libya, was conducted. All the cases of uveitis in patients under 18 years of age at diagnosis were included. The collected data included age at diagnosis, anatomical location of uveitis, laterality, associated systemic disease, used medications, and visual outcome. 75 patients (137 eyes) comprised the study sample. The mean age at the onset of uveitis was 8.9 ± 3.5 years. The female-to-male ratio was 1: 1.7. Pan uveitis was the most frequent anatomical location (54.7%), followed by anterior uveitis (36.0%), posterior (8.0%), and intermediate (1.3%). The bilateral eye was involved in 82.7%, and the unilateral eye was involved in 17.3%. The common causes of non-infectious uveitis are chronic idiopathic (52.0%), which was the most frequent etiology. Other causes associated with systemic diseases. The most frequent systemic disease was juvenile idiopathic arthritis (20.0%), frosted form-associated uveitis (10.7%), followed by Behcet disease (10.7%), HLA B27-associated uveitis (2.7%), and Vogt-Koyanagi-Harada disease (2.7%). Complications occurred in 78.8% of affected eyes. The most common complications were posterior synechia (41.3%), cataract (18.7%), glaucoma (21.3%), and cystoid macular edema (20.0%).
2026-08-08 | Adalimumab Therapy Reduces Treatment Burden in Multifocal Choroiditis/Punctate Inner Choroidopathy.
This study aims to demonstrate the efficacy of adalimumab (ADA) in reducing overall treatment burden in a cohort of patients with idiopathic multifocal choroiditis (MFC)/punctate inner choroidopathy (PIC). This is a retrospective case series of eyes classified as having idiopathic MFC/PIC using standard multi-modal imaging, including color fundus photography, fundus autofluorescence, and spectral-domain optical coherence tomography. LogMAR best-corrected visual acuity (BCVA), doses of prednisone and other immunomodulatory therapies (IMT), injections of corticosteroids or anti-vascular endothelial growth factor (anti-VEGF), and breakthrough flares were collected at baseline (initiation of ADA), 12 months from baseline, and last follow-up. Twenty-five patients (21 females, 41 eyes) were included. Mean follow-up time was 51.4 ± 35.2 months (range 9.2-126.6). Mean LogMAR BCVA was 0.35 ± 0.48 at baseline and 0.34 ± 0.55 at last follow-up (p > 0.5). Mean prednisone dosage decreased significantly from 15.76 ± 18.69 mg/day at baseline to 0.23 ± 1.1 mg/day at the last follow-up (p < 0.0001). Thirty eyes received steroid or anti-VEGF injections at baseline; this decreased to one at the last follow-up (p < 0.0001). Similarly, eight patients received other IMT at baseline, but only one continued to receive supplemental IMT at the last follow-up (p < 0.05) at a decreased dosage. Six patients experienced disease flare-up within the first 12 months of treatment, and three patients flared between 12 months and last follow-up. Adalimumab therapy demonstrated a significant steroid-sparing effect in addition to vision preservation and reduction in both anti-VEGF injections and IMT.
2026-07-17 | Demographic and socioeconomic associations with outcomes in pediatric uveitis: An IRIS® registry study
Objective To evaluate the associations of demographic and socioeconomic factors—including race and ethnicity, sex, insurance type, age, and geographic region—with visual outcomes, ocular complications, and the need for ocular surgery in children with non-infectious uveitis Design Retrospective cohort study using the American Academy of Ophthalmology IRIS® (Intelligent Research in Sight) Registry, a national ophthalmic disease registry Participants Pediatric patients with non-infectious uveitis Methods Patients with non-infectious uveitis diagnosed and treated at age ≤18 with ≥1 year follow-up with complete demographic information identified in the IRIS® Registry between January 1, 2013 and December 31, 2019. Insurance type was used as a proxy for socioeconomic status, and analyses accounting for the inclusion of both eyes of the same patient were performed using generalized estimating equations. Multivariable logistic or linear regression were performed to assess whether age, sex, race, region and insurance type were associated with higher risk of poor visual acuity outcomes, complications of uveitis and requirement for ocular surgery. Results Data from 3611 unique patients (5,723 eyes) were included. Compared to White children, Black children were more likely to have a final visual acuity of 20/200 or worse and develop glaucoma and cystoid macular edema (OR for White vs Black children for 20/200 or worse vision 0.59, 95% CI 0.42–0.82; P<0.05). Compared to children with private insurance, those with Medicaid were more likely to have a final visual acuity of 20/200 or worse, as well as suffer from amblyopia, posterior synechiae, and band keratopathy (OR for private vs Medicaid for 20/200 or worse vision 0.50, 95% CI 0.37–0.67; P<0.05 for all). Children with age ≤12 years were more likely to develop band keratopathy and amblyopia compared to those 13-18 years old (amblyopia OR 4.99, 95% CI 3.00–8.31; band keratopathy OR 2.62, 95% CI 1.89–3.63; P<0.001 for both). Conclusion Socioeconomic and demographic factors associated with poorer clinical outcomes in pediatric non-infectious uveitis include younger age at presentation, Black
2026-06-25 | Uveitis in spondyloarthritis: clinical patterns and differences between axial and peripheral forms.
The association between uveitis and spondyloarthritis is well established. However, its characterization across the different types of spondyloarthritis remains limited in the literature. Therefore, this study aimed to perform a comparative characterization of uveitis in the various types of spondyloarthritis. Retrospective observational study that included patients with non-infectious uveitis and spondyloarthritis from a multidisciplinary uveitis clinic. A descriptive analysis was performed across the different subtypes of spondyloarthritis, followed by a comparative analysis regrouping patients into axial and peripheral SpA categories. 163 patients with spondyloarthritis-associated uveitis were included, comprising radiographic axial spondyloarthritis (49%), non-radiographic axial spondyloarthritis (21%), spondyloarthritis associated with inflammatory bowel disease (13%), psoriatic arthritis (12%), and peripheral spondyloarthritis (4%). Anterior uveitis (97%), acute relapsing course (79%), and alternating laterality (47.4%) were predominant in the overall sample. When comparing axial versus peripheral forms, significant differences were observed: intermediate, posterior, and panuveitis locations occurred exclusively in the peripheral forms, which also showed higher frequencies of chronic courses (p < 0.001), bilateralism (p < 0.05), and greater use of systemic therapy (p = 0.05). Regarding this treatment to control uveitis, 28.2% of patients required immunomodulatory therapy (more sulfasalazine in axial forms, and methotrexate in peripheral) and 17.8% required biologic therapy. Biologic discontinuation was higher in peripheral forms (p = 0.059). Variables predicting greater need for systemic therapy included chronic course, bilateralism, higher number of annual episodes, younger age of onset, peripheral joint involvement, and presence of vitritis (all p < 0.05). Acute anterior uveitis constitutes the most frequent pattern in all spondyloarthritis types. However, peripheral forms exhibit a higher prevalence of non-anterior, chronic, and bilateral uveitis, and, additionally, appeared to require greater use of immunomodulatory therapy. Key Points • Understanding and distinguishing the ocular inflammatory processes associated with SpA may have important therapeutic and prognostic implications. • Although acute recurrent anterior uveitis was the most frequent patternacross all SpA subtypes, in the peripheral SpA group (pSpA, IBD-SpA, and PsA) a higher prevalence of intermediate, posterior, panuveitis, chronic course, and bilateral uveitis was observed. • The peripheral SpA group (pSpA, IBD-SpA, and PsA) appeared to require greater use of immunomodulatory drugs (csDMARDs or biologics) for control of the ocular condition, suggesting the need for closer monitoring and tailored therapeutic strategies.
2026-06-19 | Bromfenac 0.09% as adjunct to anti-VEGF for the treatment of macular edema secondary to noninfectious uveitis
Uveitis is a major cause of visual impairment and blindness and macular edema is the major risk factor. Uveitic macular edema (UME) mechanism is primarily through blood-retinal barrier breakdown driven by inflammatory mediators such as prostaglandins and vascular endothelial growth factor (VEGF). Corticosteroids are the main treatment but can cause significant side effects leading to the use of alternatives including topical non-steroidal anti-inflammatory drugs (NSAIDs) and intravitreal anti-VEGF agents. Bromfenac is an FDA-approved NSAID for postoperative inflammation but its efficacy in UME is not clear. We report a case of UME secondary to non-infectious uveitis managed with topical bromfenac in combination with intravitreal aflibercept. Case Presentation: A 13-year-old female with a history of chronic intermittent bilateral posterior uveitis presented for follow-up 6 months after stopping biologic treatment and 2 years of stable disease activity. Uncorrected visual acuity was 6/12 in both eyes (OU) and recurrent macular edema was found in the right eye (OD) with central foveal thickness (CFT) of 718 µm. The patient was started on bromfenac 0.09% twice daily and received a single intravitreal anti-VEGF injection. At one-week follow-up, the CFT decreased to 419 µm. At four-week follow-up, the CFT decreased to 318 µm OD and there was significant improvement of the macular edema.Conclusion: Bromfenac 0.09% as adjunctive to anti-VEGF injection may be an effective treatment for macular edema secondary to non-infectious uveitis.
2026-04-29 | Demographic and clinical pattern of non-infectious uveitis in the Tripoli Children's Hospital
Uveitis is relatively rare in the pediatric population, but it leads to considerable ocular morbidity. This study aims to describe the clinical, etiological, and treatment features of noninfectious uveitis in Libyan children in a pediatric rheumatology clinic. A retrospective analysis of medical records of pediatric patients who were diagnosed with noninfectious uveitis from January 2000 to December 2021 at the pediatric rheumatology clinic at Tripoli Children's Hospital, Tripoli, Libya, was conducted. All the cases of uveitis in patients under 18 years of age at diagnosis were included. The collected data included age at diagnosis, anatomical location of uveitis, laterality, associated systemic disease, used medications, and visual outcome. 75 patients (137 eyes) comprised the study sample. The mean age at the onset of uveitis was 8.9 ± 3.5 years. The female-to-male ratio was 1: 1.7. Pan uveitis was the most frequent anatomical location (54.7%), followed by anterior uveitis (36.0%), posterior (8.0%), and intermediate (1.3%). The bilateral eye was involved in 82.7%, and the unilateral eye was involved in 17.3%. The common causes of non-infectious uveitis are chronic idiopathic (52.0%), which was the most frequent etiology. Other causes associated with systemic diseases. The most frequent systemic disease was juvenile idiopathic arthritis (20.0%), frosted form-associated uveitis (10.7%), followed by Behcet disease (10.7%), HLA B27-associated uveitis (2.7%), and Vogt-Koyanagi-Harada disease (2.7%). Complications occurred in 78.8% of affected eyes. The most common complications were posterior synechia (41.3%), cataract (18.7%), glaucoma (21.3%), and cystoid macular edema (20.0%).
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Drug Discovery Landscape
8 orphan drug designations for Non-infectious posterior uveitis.
8 orphan drug designations for Non-infectious posterior uveitis.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
4-((E)-(5-(2-(2-((S)-2-((S)-1-(L-threonyl-L-lysyl)pyrrolidine-2-carboxamido)-5-guanidinopentanamido)acetamido)-2-carboxyethyl)-2-hydroxyphenyl)diazenyl)phenyl (2-(trimethylammonio)ethyl) phosphate | peptides | EMA | 2019-11-13 | — | Granzer Regulatory Consulting & Services GmbH |
DNA plasmid encoding a recombinant fusion protein consisting of the extracellular domain of human TNFα p55 receptor linked to the human IgG1 Fc domain | proteins | EMA | 2016-02-17 | — | PulseSight Therapeutics |
purified autologous type 1 regulatory T lymphocytes specific for human type II collagen | cell therapies | FDA | 2015-09-01 | — | TxCell SA |
Triamcinolone acetonide | small molecules | EMA | 2015-05-21 | — | S-cubed Pharmaceutical Services ApS |
sirolimus | small molecules | FDA | 2011-11-04 | — | Santen Inc. |
Dexamethasone | small molecules | EMA | 2010-08-04 | — | Allergan Pharmaceuticals Ireland |
secukinumab | antibodies | FDA | 2010-03-26 | — | Novartis Pharmaceutical Corporation |
Fluocinolone acetonide (prolonged-release intravitreal implant) | small molecules | EMA | 2005-03-07 | — | Bausch & Lomb Ireland |
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