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Iridociclite heterocrômica de Fuchs
ORPHA:263479CID-10 · H20.8CID-11 · 9A96.1DOENÇA RARA

A iridociclite heterocrômica de Fuchs (FHI) é uma doença ocular de etiologia desconhecida que ocorre em uma porcentagem muito pequena (0,5-6,2%) dos casos de uviete, caracterizada por heterocromia ou atrofia difusa da íris, precipitados ceráticos na ausência de sinéquias e, em alguns casos, evoluindo para glaucoma e opacidades vítreas.

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Introdução

O que você precisa saber de cara

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A iridociclite heterocrômica de Fuchs (FHI) é uma doença ocular de etiologia desconhecida que ocorre em uma porcentagem muito pequena (0,5-6,2%) dos casos de uviete, caracterizada por heterocromia ou atrofia difusa da íris, precipitados ceráticos na ausência de sinéquias e, em alguns casos, evoluindo para glaucoma e opacidades vítreas.

Publicações científicas
122 artigos
Último publicado: 2026 Mar 7

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.5
Finland
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: H20.8
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Características mais comuns

90%prev.
Precipitados ceráticos corneanos
Muito frequente (99-80%)
90%prev.
Moscas volantes
Muito frequente (99-80%)
90%prev.
Atrofia da íris
Muito frequente (99-80%)
90%prev.
Células inflamatórias da câmara anterior
Muito frequente (99-80%)
55%prev.
Heterocromia da íris
Frequente (79-30%)
55%prev.
Nódulos de Bussaca
Frequente (79-30%)
24sintomas
Muito frequente (4)
Frequente (5)
Ocasional (9)
Muito raro (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.

Precipitados ceráticos corneanosCorneal keratic precipitates
Muito frequente (99-80%)90%
Moscas volantesVitreous floaters
Muito frequente (99-80%)90%
Atrofia da írisIris atrophy
Muito frequente (99-80%)90%
Células inflamatórias da câmara anteriorAnterior chamber inflammatory cells
Muito frequente (99-80%)90%
Heterocromia da írisHeterochromia iridis
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico122PubMed
Últimos 10 anos41publicações
Pico20177 papers
Linha do tempo
2026Hoje · 2026📈 2017Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Diagnóstico

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

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Publicações mais relevantes

Timeline de publicações
42 papers (10 anos)
#1

Near-Infrared Autofluorescence in Non-Infectious Uveitis: A Review.

Ocular immunology and inflammation2026 Feb

This review offers a comprehensive synthesis of current evidence on near-infrared autofluorescence (NIR-AF) in non-infectious uveitis, highlighting its strengths, limitations, and role in diagnosis, monitoring, and understanding disease mechanisms. Unlike blue-light autofluorescence, which mainly detects lipofuscin, NIR-AF visualizes melanin and related compounds in the retinal pigment epithelium (RPE) and choroid, providing deeper penetration, reduced phototoxicity, and greater comfort. Across entities like Vogt-Koyanagi-Harada disease, MEWDS, punctate inner choroidopathy, APMPPE, and Fuchs' heterochromic iridocyclitis, NIR-AF reveals patterns often invisible on conventional imaging-detecting subclinical lesions, differentiating active from inactive disease, and tracking RPE changes over time. Its persistence in showing hypoautofluorescent or hyperautofluorescent lesions after clinical resolution offers unique insight into residual or subclinical inflammation. The technique complements OCT, fluorescein, and indocyanine green angiography, adding a melanin-specific layer to multimodal imaging. Limitations include a weaker signal compared to BL-AF, susceptibility to media opacities, equipment-dependent variability, and lack of standardized interpretation criteria. While it cannot quantify choroidal melanin loss directly and image acquisition can be challenging, its non-invasive, repeatable nature and diagnostic yield make it a promising tool for longitudinal uveitis care. Further prospective studies, standardization, and AI-driven analysis could expand its clinical impact, potentially cementing NIR-AF as an essential component in uveitis imaging strategies.

#2

Efficacy of pars plana vitrectomy for symptomatic vitreous floaters associated with Fuchs heterochromic iridocyclitis.

BMC ophthalmology2026 Mar 07
#3

Coincident Pellucid Marginal Degeneration and Fuchsiridocyclitis: a case report.

International journal of surgery case reports2025 Nov

We report a rare coexistence of Pellucid Marginal Degeneration (PMD) and Fuchs' heterochromic iridocyclitis (FHI) in a 40-year-old female. The overlap of corneal ectasia and chronic uveitis created significant diagnostic and surgical challenges, highlighting the importance of individualized planning in complex anterior segment diseases. The patient presented with painless, progressive visual decline in the right eye. Clinical examination revealed inferior corneal thinning, mature cataract, pigmented keratic precipitates, and peripheral anterior synechiae (PAS). Corneal tomography confirmed PMD with a characteristic "crab-claw" pattern. The patient underwent phacoemulsification with posterior chamber intraocular lens implantation. Postoperatively, visual acuity improved, although optic disc cupping and persistent PAS underscored the need for long-term glaucoma surveillance. The coexistence of PMD and FHI is exceptionally rare. PMD complicates surgical planning by affecting keratometric reliability and corneal biomechanics, while FHI contributes to cataract formation, intraocular inflammation, and risk of secondary glaucoma. Multimodal imaging, tailored surgical strategy, and vigilant postoperative follow-up are key to optimizing outcomes. Simultaneous PMD and FHI represent a unique clinical entity requiring comprehensive assessment and individualized management. Tailored surgical planning and vigilant surveillance are essential to preserve vision and mitigate long-term risks.

#4

Ultrasound biomicroscopic findings in Fuchs uveitis syndrome: A contralateral eye study.

Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society2025

The aim of this study was to determine ultrasound biomicroscopic findings in patients with Fuchs uveitis syndrome (FUS). This cross-sectional, contralateral comparative eye study was conducted on patients with unilateral FUS. Both eyes of each patient underwent ultrasound biomicroscopy (UBM) imaging at the 12, 3, 6, and 9-o'clock radial meridians. Ultrasonographic data were collected, including the thickness (mm) of the ciliary body (CB), CB with ciliary processes, and iris thickness at 0.8 mm from the iris root, mid-iris, and iris tip. We enrolled 36 eyes from 18 patients with unilateral FUS, including 10 (55.6%) females, with a mean age of 38.44 ± 9.2 years. The average thickness of CB (P < 0.001), 0.8 mm from the iris root (P = 0.003), mid-iris (P < 0.001), and the iris tip (P < 0.001) was thinner in the affected eyes compared to the fellow eyes of the patients. Analysis of variance analysis showed that differences among all quadrants were not significant in both groups (P > 0.05). This study showed that CB and iris thickness were significantly thinner in the affected eyes compared to the fellow eyes of patients with FUS, as assessed using the UBM technique.

#5

Retinal and choroidal thickness in fuchs uveitis syndrome: a contralateral eye study.

BMC ophthalmology2024 Jul 12

To investigate the subfoveal retinal and choroidal thickness in patients with unilateral Fuchs Uveitis Syndrome (FUS). This comparative contralateral study was performed in affected eyes with FUS versus fellow eyes. For each eye parameters such as subfoveal choroidal thickness (SCT), subfoveal choriocapillary thickness (SCCT), central macular thickness (CMT), and central macular volume (CMV) were measured; then the measured values of affected and fellow unaffected eye were compared. Thirty-seven patients (74 eyes) including 19 females (51.4%) with a mean age of 36.9 ± 7.6 years were enrolled. The mean SCT was lower in the affected eyes (344.51 ± 91.67) than in the fellow (375.59 ± 87.33) with adjusting for duration of disease and axial lengths (P < 0.001). The mean SCCT, CMT, and CMV were higher in eyes with FUS than in fellow eyes (P < 0.05). The result of our study demonstrated that affected eyes in patients with FUS tend to have thinner SCT and thicker SCCT and CMT compared to uninvolved fellow eyes.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC71 artigos no totalmostrando 40

2026

Efficacy of pars plana vitrectomy for symptomatic vitreous floaters associated with Fuchs heterochromic iridocyclitis.

BMC ophthalmology
2026

Near-Infrared Autofluorescence in Non-Infectious Uveitis: A Review.

Ocular immunology and inflammation
2025

Coincident Pellucid Marginal Degeneration and Fuchsiridocyclitis: a case report.

International journal of surgery case reports
2024

Retinal and choroidal thickness in fuchs uveitis syndrome: a contralateral eye study.

BMC ophthalmology
2024

Fuchs' uveitis syndrome: a 20-year experience in 466 patients.

Scientific reports
2024

Association of Fuchs Heterochromic Iridocyclitis with Multiple Sclerosis.

Ceska a slovenska oftalmologie : casopis Ceske oftalmologicke spolecnosti a Slovenske oftalmologicke spolecnosti
2025

Ultrasound biomicroscopic findings in Fuchs uveitis syndrome: A contralateral eye study.

Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society
2024

Multiple sclerosis-associated uveitis - A series from South India.

Indian journal of ophthalmology
2022

Factors associated with low prevalence of Fuchs' uveitis syndrome in Japan.

Frontiers in medicine
2022

Treatment of Complications Due to Fuchs Heterochromic Iridocyclitis (FHI) - a Case Report.

Acta informatica medica : AIM : journal of the Society for Medical Informatics of Bosnia &amp; Herzegovina : casopis Drustva za medicinsku informatiku BiH
2023

XEN® 63 gel stent to treat a refractory uveitic glaucoma: A case report.

European journal of ophthalmology
2022

Optic coherence tomography angiography findings in fuchs heterochromic iridocyclitis.

International ophthalmology
2022

Cytokine Changes in the Aqueous Humor in Rubella-Related Fuchs Heterochromic Iridocyclitis.

Disease markers
2022

Novel disease-causing variants and phenotypic features of X-linked megalocornea.

Acta ophthalmologica
2021

Pattern of Uveitis in Iran: A Systematic Review.

Journal of ophthalmic &amp; vision research
2020

Comments on: Corneal endothelium in unilateral Fuchs heterochromic iridocyclitis.

Indian journal of ophthalmology
2020

Polychromasia capsulare in Fuchs heterochromic iridocyclitis: case report and literature review.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2020

Corneal endothelium in unilateral Fuchs heterochromic iridocyclitis.

Indian journal of ophthalmology
2020

Analysis of retinal microvasculature in Fuchs' uveitis syndrome. Retinal microvasculature in Fuchs' uveitis.

Journal francais d'ophtalmologie
2020

A case of bilateral acute depigmentation of the Iris in one of two identical twins.

BMC ophthalmology
2020

The outcome of initial mitomycin C-augmented trabeculectomy with subconjunctival bevacizumab in the management of secondary glaucoma associated with Fuchs heterochromic iridocyclitis.

International ophthalmology
2021

Clinical Features and Diagnosis of Anterior Segment Inflammation Related to Cytomegalovirus in Immunocompetent African, Asian, and Caucasian Patients.

Ocular immunology and inflammation
2019

Cornea Findings of Spectral Domain Anterior Segment Optical Coherence Tomography in Uveitic Eyes of Various Etiologies.

Cornea
2019

Iris nodules in Fuchs heterochromic iridocyclitis.

Indian journal of ophthalmology
2019

Predictive Factors of Intraocular Pressure Level Evolution Over Time and Glaucoma Severity in Fuchs' Heterochromic Iridocyclitis.

Investigative ophthalmology &amp; visual science
2019

Association of Ocular Inflammation and Rubella Virus Persistence.

JAMA ophthalmology
2018

Moth-eaten appearance of the iris in Fuchs heterochromic iridocyclitis.

Journal francais d'ophtalmologie
2018

Fuchs heterochromic iridocyclitis-associated glaucoma: a retrospective comparison of primary Ahmed glaucoma valve implantation and trabeculectomy with mitomycin C.

F1000Research
2017

Niemann-Pick Disease Type C Associated with Fuchs Heterochromic Iridocyclitis.

Advanced biomedical research
2017

Review of Epidemiology of Uveitis in Asia: Pattern of Uveitis in a Tertiary Hospital in the Philippines.

Ocular immunology and inflammation
2017

Outcomes of phacoemulsification in patients with uveitis at a tertiary center in São Paulo, Brazil: a review of cases from 2007 to 2012.

Arquivos brasileiros de oftalmologia
2017

Topical Ganciclovir in Cytomegalovirus Anterior Uveitis.

Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics
2017

The unusual association of inverse retinitis pigmentosa and Fuchs' heterochromic iridocyclitis.

International journal of retina and vitreous
2016

Fuchs' Heterochromic Iridocyclitis in an Italian Tertiary Referral Centre: Epidemiology, Clinical Features, and Prognosis.

Journal of ophthalmology
2017

Risk Factors for Glaucoma in a Cohort of Patients with Fuchs Heterochromic Iridocyclitis.

Ocular immunology and inflammation
2016

Phacoemulsification Versus Manual Small Incision Cataract Surgery in Patients With Fuchs Heterochromic Iridocyclitis.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2017

Uveitis in São Paulo, Brazil: 1053 New Patients in 15 Months.

Ocular immunology and inflammation
2015

Bilateral uveitis and Usher syndrome: a case report.

Journal of medical case reports
2015

In-bag dislocation of intraocular lens in patients with uveitis: a case series.

Journal of ophthalmic inflammation and infection
2014

Small-incision cataract surgery in patients with Fuch's heterochromic iridocyclitis.

Nepalese journal of ophthalmology : a biannual peer-reviewed academic journal of the Nepal Ophthalmic Society : NEPJOPH
Ver todos os 71 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Near-Infrared Autofluorescence in Non-Infectious Uveitis: A Review.
    Ocular immunology and inflammation· 2026· PMID 41417717mais citado
  2. Efficacy of pars plana vitrectomy for symptomatic vitreous floaters associated with Fuchs heterochromic iridocyclitis.
    BMC ophthalmology· 2026· PMID 41794659mais citado
  3. Coincident Pellucid Marginal Degeneration and Fuchsiridocyclitis: a case report.
    International journal of surgery case reports· 2025· PMID 41332050mais citado
  4. Ultrasound biomicroscopic findings in Fuchs uveitis syndrome: A contralateral eye study.
    Saudi journal of ophthalmology : official journal of the Saudi Ophthalmological Society· 2025· PMID 41367835mais citado
  5. Retinal and choroidal thickness in fuchs uveitis syndrome: a contralateral eye study.
    BMC ophthalmology· 2024· PMID 38997641mais citado
  6. Fuchs' uveitis syndrome: a 20-year experience in 466 patients.
    Sci Rep· 2024· PMID 38616214recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:263479(Orphanet)
  2. MONDO:0016989(MONDO)
  3. GARD:6791(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q3676446(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Iridociclite heterocrômica de Fuchs
Compêndio · Raras BR

Iridociclite heterocrômica de Fuchs

ORPHA:263479 · MONDO:0016989
Prevalência
1-9 / 1 000 000
CID-10
H20.8 · Outras iridociclites
CID-11
Início
Adult
Prevalência
0.5 (Finland)
MedGen
UMLS
C0016782
Repurposing
2 candidatos
prednisoloneglucocorticoid receptor agonist
prednisolone-acetate
EuropePMC
Wikidata
Papers 10a
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