RARE DISEASE
Intermediate uveitis
Intermediate uveitis
Intermediate uveitis
Synonyms: IU
Synonyms: IU
Synonyms: IU
Drug discovery
4
drugs
With orphan designations
Overview
Intermediate Uveitis (IU) is chronic inflammation predominantly affecting the vitreous and peripheral retina, defined by Standardization of Uveitis Nomenclature (SUN) criteria. While often idiopathic, adult cases may associate with multiple sclerosis or sarcoidosis. Pediatric IU (15-33% of childhood uveitis) frequently presents bilaterally with risks of macular edema (36%) and cataracts. Symptoms include floaters, blurred vision, and photophobia. Treatment combines corticosteroids with immunomodulators, though recurrence rates remain high, emphasizing early intervention [1][4][7][17].
Therapies
Stepwise approach: periocular/intravitreal corticosteroids → systemic steroids → immunomodulators (methotrexate, mycophenolate) → biologics (adalimumab) [2][3][8]
Surgical interventions (vitrectomy, cataract extraction) for complications [2][7]
61–85% achieve inflammation control with immunomodulators; biologics reduce relapse risk [3][8][18]
Categories: rare ophthalmic disorders
Research Papers
697 drug discovery papers about Intermediate uveitis, with 2 first-in-class and 1 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
697 drug discovery papers about Intermediate uveitis, with 2 first-in-class and 1 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
2026-06-13 | Real-World Anatomical Outcomes of Suprachoroidal Triamcinolone Acetonide Injections in a Large Retinal Practice over a 4-Year Period.
To evaluate the clinical role, durability, anatomical response, and safety for suprachoroidal triamcinolone acetonide (SCTA) (Xipere) in routine clinical practice. Data were manually extracted from health records at a high-volume retina practice in Cleveland, Ohio. All patients with noninfectious inflammatory macular edema undergoing SCTA (≥1 billing code) between October 25, 2021, and July 17, 2025, were included. Patients with known systemic autoimmune or infectious associations were excluded. Unique eyes were defined by MRN + laterality, with "OU" entries split into OD/OS. Patient demographics, underlying diagnosis categorization, time between first and second injection (for eyes with ≥2 injections) were summarized. Pre- versus post-treatment central retinal thickness (CRT) and intraocular pressure (IOP) were compared via paired t-tests. A total of 177 patients (195 eyes) received 340 SCTA injections for macular edema associated with an underlying diagnosis of intermediate uveitis (10%), pseudophakic cystoid macular edema (31%), and posterior uveitis (59%). The time between first and second injection was approximately 5 months [SD ± 72.5; median 144 (min 35, max 427)]. From baseline to follow-up, mean CRT decreased by 103 µm [95% CI: (-122,-83.2), P < 0.001], while IOP increased minimally [mean difference 0.7 mmHg, 95% CI: (-0.02, 1.43), P = 0.057]; and was medically managed when elevated. In this real-world cohort, findings suggest that SCTA achieves robust anatomical improvement, a favorable IOP profile, and prolonged durability across patients with inflammation-related macular edema.
2026-06-02 | Granulomatous Panuveitis Associated with Body Piercing
To report a case of bilateral granulomatous panuveitis temporally associated with a body piercing and to highlight a potential immune-mediated mechanism analogous to tattoo-associated uveitis. This case included comprehensive clinical evaluation, multimodal ophthalmic imaging, and laboratory testing for infectious and systemic autoimmune disease. Treatments included corticosteroids and immunomodulatory therapy. A 16-year-old female with no past medical or ocular history and no history of tattoos developed progressive bilateral vision loss shortly after navel piercing placement. Her visual acuity was 20/200 in the right eye and hand motion in the left eye. Examination revealed granulomatous keratic precipitates, posterior synechiae, cataracts, vitritis, and disc edema, consistent with bilateral granulomatous panuveitis. Optical coherence tomography revealed diffuse intraretinal fluid, subretinal fluid, and bacillary detachment with a thick choroid. Fluorescein angiography showed disc leakage, macular leakage, and diffuse perivascular leakage. Intermediate-late-phase indocyanine green angiography demonstrated patchy hypofluorescent dark dots. Extensive infectious and systemic autoimmune work-up was negative. Her review of systems was negative. The patient achieved marked improvement and durable inflammation control with high doses of corticosteroids, methotrexate, and infliximab. We describe a case of bilateral granulomatous panuveitis temporally associated with a body piercing; however, causality cannot be established, and the proposed mechanism remains hypothesis-generating. Metal-specific delayed-type hypersensitivity or foreign body granulomatous inflammation, also implicated in tattoo-associated granulomatous uveitis, represents a plausible mechanism. Clinicians should inquire about recent tattoos or piercings when evaluating bilateral granulomatous uveitis of unclear origin, as early recognition and appropriate immunomodulatory therapy may prevent vision loss and morbidity.
2026-05-28 | Atypical COQ2-Related Retinopathy in Identical Twins with Nephropathy Mimicking Intermediate Uveitis.
To describe an atypical presentation of COQ2-related retinopathy in identical twins with nephropathy, mimicking intermediate uveitis with cystoid macular oedema (CMO). Retrospective case report. A 33-year-old man presented with bilateral vision worsening and suspected intermediate uveitis. Examination revealed vitreous cells, CMO, retinal microangiopathy, and severely abnormal electrooculography (EOG) with only borderline full-field electroretinography (ERG) changes. CMO worsened with topical corticosteroids but improved bilaterally after a single unilateral intravitreal bevacizumab injection, suggesting a systemic therapeutic effect. His identical twin exhibited very similar retinal and systemic findings. Whole-exome sequencing identified a homozygous likely pathogenic COQ2 variant (c.683A > G), confirming primary coenzyme Q10 (CoQ10) deficiency type 1. Both twins also had nephropathy consistent with focal segmental glomerulosclerosis (FSGS) but no neurological involvement. This report expands the phenotypic spectrum of COQ2-related retinopathy, characterized by retinal microangiopathy, CMO, and primary retinal pigment epithelium (RPE) dysfunction with preserved rod function, in contrast to the typical retinitis pigmentosa-like phenotype. Recognition of this presentation is critical, as early CoQ10 supplementation may stabilize disease progression and prevent systemic complications. Genetic testing should be considered in young patients with CMO resembling intermediate uveitis, particularly when associated with nephropathy.
2026-04-01 | Culprit and cure: Spontaneous closure of an inflammation-induced macular hole
A 59-year-old female presented with blurring of vision in OS for 1 year. She had been diagnosed to have intermediate uveitis due to sarcoidosis and received two doses of posterior subtenon injection of triamcinolone. On presentation to us, the best corrected visual acuity (BCVA) was 6/7.5 in OD and 2/60 in OS. Anterior segment examination revealed occasional cells and flare 1+ in and cells in anterior vitreous bilaterally. Fundus examination showed media haze due to vitritis [Fig. 1a and b], hyperemic disc, macular edema (CME) in OU with full thickness macular hole (MH) with a base diameter of 1023 µm, apex diameter 179 µm, and hole height 570 µm was observed in OS [Fig. 1b] by optical coherence tomography (OCT) [Fig. 1c]. Investigations showed alarmingly poor glycemic control. Intravitreal dexamethasone implant was administered in the left eye, and oral methotrexate (15 mg/week) was initiated. After 2 months, the visual acuity in OD improved to 6/6 and OS to 6/60 with improvement in inflammation in OU. Macular edema in OD resolved, while OS showed gradual anatomical improvement in OCT [Fig. 1d]. At 5 months, the MH had closed [Fig. 1e] and BCVA improved to 6/18. There was complete resolution of inflammation with immunosuppression, and oral methotrexate was continued. OCT at 10 months showing closure of hole with loss of ellipsoid zone layer and external limiting membrane [Fig. 1f].Figure 1: (a, b): Fundus photograph of right eye showing normal disc with cystic macula and left eye showing macular hole at presentation. (c): Left eye OCT at presentation showing full thickness macular hole with cystoid macular edema. (d): OCT at 2 months showing approximation of inner retinal layers with inner retinal cystic spaces. (e): OCT at 3 months showing inner layer closure with persistent outer layer defect. (f): OCT at 10 months showing closure of hole with loss of ellipsoid zone layer and external limiting membraneDiscussion The index case highlights that clinicians should exercise caution when managing MH in uveitis patients as those secondary to inflammatory CME may not always require surgical intervention.[1] The retinal thickening and increased volume caused by CME, which brings the edges of the MH closer together, result in closure due to primary intention. This proximity may facilitate the natural formation of a tissue “bridge” (often via glial or Müller cell proliferation) across the defect, allowing MH to close without surgical intervention.[2] According to a study by Wang et al., holes under 200 µm size have a substantial chance of MH closure with medical therapy. In the absence of anteroposterior traction, macular dehydration occurs and hole edges reoppose.[3,4] Authors’ contributions Dr. RK prepared the draft, Dr. SP collected the data, and images Dr. PDM was involved in patient management, provided additional inputs and finalized the manuscript. All the authors reviewed the final manuscript. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.
2026-04-01 | Longitudinal Follow-Up of Peripapillary Kyrieleis Plaques in a Case with Unilateral Intermediate Uveitis Related to Ocular Toxoplasmosis without a Focus of Retinochoroiditis.
To describe an atypical presentation of ocular toxoplasmosis, characterized by dense vitritis and circumferential peripapillary Kyrieleis plaques. Case report. A 49-year-old healthy man presented with right visual deterioration. He had previously received a short course oral corticosteroid treatment without systemic evaluation. On examination, dense vitritis and peripapillary, near-360 degrees Kyrieleis plaques without any focus of chorioretinitis were noted. Fluorescein angiography showed diffuse posterior pole masking from vitritis and mild late optic disc hyperfluorescence without peripheral vascular leakage. Infectious screening showed positive Toxoplasma gondii (IgG) and negative IgM. A diagnosis of ocular toxoplasmosis-related unilateral intermediate uveitis was established. Oral trimethoprim/sulfamethoxazole and azithromycin were initiated, resulting in improved visual acuity and reduced vitritis within three weeks. Five months later, new visual decline due to macular edema was treated with oral methylprednisolone and trimethoprim/sulfamethoxazole, leading to resolution. Over 10 months, Kyrieleis plaques decreased but did not fully disappear. Ocular toxoplasmosis can manifest atypically without a focus of retinochoroiditis. Kyrieleis plaques should prompt consideration of infectious causes before corticosteroid therapy.
2026-06-13 | Real-World Anatomical Outcomes of Suprachoroidal Triamcinolone Acetonide Injections in a Large Retinal Practice over a 4-Year Period.
To evaluate the clinical role, durability, anatomical response, and safety for suprachoroidal triamcinolone acetonide (SCTA) (Xipere) in routine clinical practice. Data were manually extracted from health records at a high-volume retina practice in Cleveland, Ohio. All patients with noninfectious inflammatory macular edema undergoing SCTA (≥1 billing code) between October 25, 2021, and July 17, 2025, were included. Patients with known systemic autoimmune or infectious associations were excluded. Unique eyes were defined by MRN + laterality, with "OU" entries split into OD/OS. Patient demographics, underlying diagnosis categorization, time between first and second injection (for eyes with ≥2 injections) were summarized. Pre- versus post-treatment central retinal thickness (CRT) and intraocular pressure (IOP) were compared via paired t-tests. A total of 177 patients (195 eyes) received 340 SCTA injections for macular edema associated with an underlying diagnosis of intermediate uveitis (10%), pseudophakic cystoid macular edema (31%), and posterior uveitis (59%). The time between first and second injection was approximately 5 months [SD ± 72.5; median 144 (min 35, max 427)]. From baseline to follow-up, mean CRT decreased by 103 µm [95% CI: (-122,-83.2), P < 0.001], while IOP increased minimally [mean difference 0.7 mmHg, 95% CI: (-0.02, 1.43), P = 0.057]; and was medically managed when elevated. In this real-world cohort, findings suggest that SCTA achieves robust anatomical improvement, a favorable IOP profile, and prolonged durability across patients with inflammation-related macular edema.
2026-06-02 | Granulomatous Panuveitis Associated with Body Piercing
To report a case of bilateral granulomatous panuveitis temporally associated with a body piercing and to highlight a potential immune-mediated mechanism analogous to tattoo-associated uveitis. This case included comprehensive clinical evaluation, multimodal ophthalmic imaging, and laboratory testing for infectious and systemic autoimmune disease. Treatments included corticosteroids and immunomodulatory therapy. A 16-year-old female with no past medical or ocular history and no history of tattoos developed progressive bilateral vision loss shortly after navel piercing placement. Her visual acuity was 20/200 in the right eye and hand motion in the left eye. Examination revealed granulomatous keratic precipitates, posterior synechiae, cataracts, vitritis, and disc edema, consistent with bilateral granulomatous panuveitis. Optical coherence tomography revealed diffuse intraretinal fluid, subretinal fluid, and bacillary detachment with a thick choroid. Fluorescein angiography showed disc leakage, macular leakage, and diffuse perivascular leakage. Intermediate-late-phase indocyanine green angiography demonstrated patchy hypofluorescent dark dots. Extensive infectious and systemic autoimmune work-up was negative. Her review of systems was negative. The patient achieved marked improvement and durable inflammation control with high doses of corticosteroids, methotrexate, and infliximab. We describe a case of bilateral granulomatous panuveitis temporally associated with a body piercing; however, causality cannot be established, and the proposed mechanism remains hypothesis-generating. Metal-specific delayed-type hypersensitivity or foreign body granulomatous inflammation, also implicated in tattoo-associated granulomatous uveitis, represents a plausible mechanism. Clinicians should inquire about recent tattoos or piercings when evaluating bilateral granulomatous uveitis of unclear origin, as early recognition and appropriate immunomodulatory therapy may prevent vision loss and morbidity.
2026-05-28 | Atypical COQ2-Related Retinopathy in Identical Twins with Nephropathy Mimicking Intermediate Uveitis.
To describe an atypical presentation of COQ2-related retinopathy in identical twins with nephropathy, mimicking intermediate uveitis with cystoid macular oedema (CMO). Retrospective case report. A 33-year-old man presented with bilateral vision worsening and suspected intermediate uveitis. Examination revealed vitreous cells, CMO, retinal microangiopathy, and severely abnormal electrooculography (EOG) with only borderline full-field electroretinography (ERG) changes. CMO worsened with topical corticosteroids but improved bilaterally after a single unilateral intravitreal bevacizumab injection, suggesting a systemic therapeutic effect. His identical twin exhibited very similar retinal and systemic findings. Whole-exome sequencing identified a homozygous likely pathogenic COQ2 variant (c.683A > G), confirming primary coenzyme Q10 (CoQ10) deficiency type 1. Both twins also had nephropathy consistent with focal segmental glomerulosclerosis (FSGS) but no neurological involvement. This report expands the phenotypic spectrum of COQ2-related retinopathy, characterized by retinal microangiopathy, CMO, and primary retinal pigment epithelium (RPE) dysfunction with preserved rod function, in contrast to the typical retinitis pigmentosa-like phenotype. Recognition of this presentation is critical, as early CoQ10 supplementation may stabilize disease progression and prevent systemic complications. Genetic testing should be considered in young patients with CMO resembling intermediate uveitis, particularly when associated with nephropathy.
2026-04-01 | Culprit and cure: Spontaneous closure of an inflammation-induced macular hole
A 59-year-old female presented with blurring of vision in OS for 1 year. She had been diagnosed to have intermediate uveitis due to sarcoidosis and received two doses of posterior subtenon injection of triamcinolone. On presentation to us, the best corrected visual acuity (BCVA) was 6/7.5 in OD and 2/60 in OS. Anterior segment examination revealed occasional cells and flare 1+ in and cells in anterior vitreous bilaterally. Fundus examination showed media haze due to vitritis [Fig. 1a and b], hyperemic disc, macular edema (CME) in OU with full thickness macular hole (MH) with a base diameter of 1023 µm, apex diameter 179 µm, and hole height 570 µm was observed in OS [Fig. 1b] by optical coherence tomography (OCT) [Fig. 1c]. Investigations showed alarmingly poor glycemic control. Intravitreal dexamethasone implant was administered in the left eye, and oral methotrexate (15 mg/week) was initiated. After 2 months, the visual acuity in OD improved to 6/6 and OS to 6/60 with improvement in inflammation in OU. Macular edema in OD resolved, while OS showed gradual anatomical improvement in OCT [Fig. 1d]. At 5 months, the MH had closed [Fig. 1e] and BCVA improved to 6/18. There was complete resolution of inflammation with immunosuppression, and oral methotrexate was continued. OCT at 10 months showing closure of hole with loss of ellipsoid zone layer and external limiting membrane [Fig. 1f].Figure 1: (a, b): Fundus photograph of right eye showing normal disc with cystic macula and left eye showing macular hole at presentation. (c): Left eye OCT at presentation showing full thickness macular hole with cystoid macular edema. (d): OCT at 2 months showing approximation of inner retinal layers with inner retinal cystic spaces. (e): OCT at 3 months showing inner layer closure with persistent outer layer defect. (f): OCT at 10 months showing closure of hole with loss of ellipsoid zone layer and external limiting membraneDiscussion The index case highlights that clinicians should exercise caution when managing MH in uveitis patients as those secondary to inflammatory CME may not always require surgical intervention.[1] The retinal thickening and increased volume caused by CME, which brings the edges of the MH closer together, result in closure due to primary intention. This proximity may facilitate the natural formation of a tissue “bridge” (often via glial or Müller cell proliferation) across the defect, allowing MH to close without surgical intervention.[2] According to a study by Wang et al., holes under 200 µm size have a substantial chance of MH closure with medical therapy. In the absence of anteroposterior traction, macular dehydration occurs and hole edges reoppose.[3,4] Authors’ contributions Dr. RK prepared the draft, Dr. SP collected the data, and images Dr. PDM was involved in patient management, provided additional inputs and finalized the manuscript. All the authors reviewed the final manuscript. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.
2026-04-01 | Longitudinal Follow-Up of Peripapillary Kyrieleis Plaques in a Case with Unilateral Intermediate Uveitis Related to Ocular Toxoplasmosis without a Focus of Retinochoroiditis.
To describe an atypical presentation of ocular toxoplasmosis, characterized by dense vitritis and circumferential peripapillary Kyrieleis plaques. Case report. A 49-year-old healthy man presented with right visual deterioration. He had previously received a short course oral corticosteroid treatment without systemic evaluation. On examination, dense vitritis and peripapillary, near-360 degrees Kyrieleis plaques without any focus of chorioretinitis were noted. Fluorescein angiography showed diffuse posterior pole masking from vitritis and mild late optic disc hyperfluorescence without peripheral vascular leakage. Infectious screening showed positive Toxoplasma gondii (IgG) and negative IgM. A diagnosis of ocular toxoplasmosis-related unilateral intermediate uveitis was established. Oral trimethoprim/sulfamethoxazole and azithromycin were initiated, resulting in improved visual acuity and reduced vitritis within three weeks. Five months later, new visual decline due to macular edema was treated with oral methylprednisolone and trimethoprim/sulfamethoxazole, leading to resolution. Over 10 months, Kyrieleis plaques decreased but did not fully disappear. Ocular toxoplasmosis can manifest atypically without a focus of retinochoroiditis. Kyrieleis plaques should prompt consideration of infectious causes before corticosteroid therapy.
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Drug Discovery Landscape
4 orphan drug designations for Intermediate uveitis.
4 orphan drug designations for Intermediate uveitis.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
tabirafusp alfa | proteins | FDA | 2025-03-04 | — | Kodiak Sciences Inc. |
vamikibart | antibodies | FDA | 2022-08-15 | — | Genentech, Inc |
Secukinumab [Cosantix] | antibodies | EMA | 2010-02-02 | — | Novartis Europharm Limited |
Voclosporin [Luveniq] | small molecules | EMA | 2007-09-14 | — | [INACTIVE] Lux Biosciences GmbH |
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