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Doença de Eales
ORPHA:40923CID-10 · H35.0CID-11 · 9B77DOENÇA RARA

A Doença de Eales (DE) é uma condição inflamatória, de causa desconhecida, que afeta e obstrui as veias da retina (a parte de trás do olho). Ela se desenvolve em três fases: inflamação dos vasos sanguíneos (vasculite), bloqueio desses vasos e a formação de novos vasos sanguíneos anormais na retina. Isso pode levar a sangramentos repetidos dentro do olho (no vítreo) e à perda de visão.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A Doença de Eales (DE) é uma condição inflamatória, de causa desconhecida, que afeta e obstrui as veias da retina (a parte de trás do olho). Ela se desenvolve em três fases: inflamação dos vasos sanguíneos (vasculite), bloqueio desses vasos e a formação de novos vasos sanguíneos anormais na retina. Isso pode levar a sangramentos repetidos dentro do olho (no vítreo) e à perda de visão.

Publicações científicas
315 artigos
Último publicado: 2026 Mar 27

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adolescent
+ adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: H35.0
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

👁️
Olhos
12 sintomas
🧠
Neurológico
3 sintomas
❤️
Coração
2 sintomas
🫃
Digestivo
1 sintomas
👂
Ouvidos
1 sintomas

+ 12 sintomas em outras categorias

Características mais comuns

55%prev.
Vasculite retiniana
Frequente (79-30%)
55%prev.
Hemorragia vítrea
Frequente (79-30%)
55%prev.
Cefaleia
Frequente (79-30%)
17%prev.
Acuidade visual reduzida
Ocasional (29-5%)
17%prev.
Cegueira
Ocasional (29-5%)
17%prev.
Descolamento de retina regmatogênico
Ocasional (29-5%)
31sintomas
Frequente (3)
Ocasional (22)
Muito raro (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 31 características clínicas mais associadas, ordenadas por frequência.

Vasculite retinianaRetinal vasculitis
Frequente (79-30%)55%
Hemorragia vítreaVitreous hemorrhage
Frequente (79-30%)55%
CefaleiaHeadache
Frequente (79-30%)55%
Acuidade visual reduzidaReduced visual acuity
Ocasional (29-5%)17%
CegueiraBlindness
Ocasional (29-5%)17%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico315PubMed
Últimos 10 anos71publicações
Pico201610 papers
Linha do tempo
2026Hoje · 2026🧪 2006Primeiro ensaio clínico📈 2016Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Doença de Eales

🗺️

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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

1 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
68 papers (10 anos)
#1

Bilateral Eales' Disease Managed With Vitrectomy and Anti-VEGF Therapy: A Case Report.

The American journal of case reports2026 Mar 05

BACKGROUND Eales' disease - a rare idiopathic retinal vasculitis typically affecting young adult men - is characterized by periphlebitis, retinal ischemia, and neovascularization. Vitreous hemorrhage and tractional retinal detachment often occur; timely diagnosis and individualized management remain critical. This report describes bilateral Eales' disease with an asymmetrical clinical course and complex treatment strategy incorporating vitrectomy, systemic immunosuppression, and targeted anti-vascular endothelial growth factor (anti-VEGF) therapy. CASE REPORT A 32-year-old White man without systemic illness, infectious exposure, or substance use exhibited left-eye vitreous hemorrhage. Best-corrected visual acuity (BCVA) was hand motion (≈2.3 logarithm of the minimum angle of resolution [logMAR]) in the left eye and 5/6 (≈0.08 logMAR) in the right eye. Ultrasonography and fluorescein angiography demonstrated vitreoretinal traction and peripheral retinal ischemia. After exclusion of infectious etiologies, the diagnosis was bilateral Eales' disease. We performed left-eye vitrectomy with cataract extraction, panretinal photocoagulation (PRP), and systemic immunosuppression with azathioprine, followed by methotrexate. One year later, right-eye disease progression required intravenous methylprednisolone, PRP, and preoperative intravitreal faricimab, followed by vitrectomy. Faricimab-mediated inhibition of vascular endothelial growth factor A (VEGF-A) and angiopoietin-2 (Ang-2) enhanced vascular stabilization. At final follow-up - 18 months after left-eye vitrectomy and 12 months after right-eye vitrectomy - BCVA was 0.00 logMAR (5/5) in both eyes. CONCLUSIONS Early vitrectomy with systemic immunosuppression and adjunctive anti-VEGF therapy may achieve excellent long-term visual outcomes in bilateral Eales' disease. Faricimab application highlights the utility of dual VEGF-A/Ang-2 pathway inhibition in surgical planning. Future studies will clarify anti-VEGF agent efficacies in this rare retinal vasculitis.

#2

Combination of Methotrexate and Mycophenolate mofetil for Refractory Uveitis & Scleritis: A Case Series.

Retinal cases &amp; brief reports2026 Feb 03

This study analyses the efficacy and safety of combination of methotrexate and mycophenolate mofetil with treatment-resistant uveitis and scleritis. Method: Retrospective Chart Review. The study included five patients with uveitis and one with scleritis who received combination of antimetabolites. The median age of these patients was 19.5 years, and all of them had bilateral involvement. Two patients had Juvenile Idiopathic Arthritis while one patient each had Behçet's disease, Eales disease, undifferentiated anterior scleritis and pars planitis. The median duration of immunosuppressive and/or corticosteroid treatment before switching to combined therapy was 217 (192-1325) days. Remission of uveitis was achieved in all patients. The median duration of follow-up in these patients was 338.5 (209-901) days. At the end of the follow-up, topical steroids were withdrawn in three eyes, and oral corticosteroids were tapered off completely. All except one patient tolerated the combined treatment. The combination of oral methotrexate and mycophenolate mofetil can be a useful alternative in patients resistant to conventional immunosuppression.

#3

Clinical trials and quasi-experimental studies in the treatment of noninfectious retinal vasculitis: A systematic review from the International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) - Report 4.

Survey of ophthalmology2026

This systematic review evaluates clinical trials and quasi-experimental studies reporting diagnostic criteria, disease activity scoring systems (including Disease Activity Index (DAI), Total Inflammatory Activity Index (TIAI), and Total Adjusted Disease Activity Index (TADAI), and others), and therapeutic strategies used in noninfectious retinal vasculitis (RV), aiming to consolidate current evidence on treatment response and effectiveness measures. Of 5533 articles screened (PROSPERO: CRD42023489232), 15 studies met the inclusion criteria. Most were conducted in Asia (73.3 %) and focused on Behçet disease (60 %) or Eales disease (27 %). RV was diagnosed clinically in all studies, while half incorporated fluorescein angiography to confirm vascular inflammation through leakage, staining, or occlusion; however, definitions of RV were highly variable, often inferred rather than explicitly stated, and lacked standardization. This diagnostic inconsistency, combined with limited imaging data, undermines comparability across studies. Disease activity was assessed using outdated composite indices such as the DAI, TIAI, and TADAI, which lack external validation and fail to capture key clinical features such as macular ischemia or capillary non-perfusion. Best corrected visual acuity, although frequently reported, was confounded by unrelated factors such as cataracts or macular scarring. Therapeutic strategies included systemic corticosteroids, immunosuppressants (e.g., methotrexate, azathioprine), and biologics (e.g., infliximab, interferon-alpha). Treatment selection was heterogeneous, and several regimens-such as cyclophosphamide-azathioprine combinations-do not align with current standards of care. Due to variability in study design and outcome reporting, no pooled effect estimates, or statistical comparisons were conducted. Treatment outcomes were synthesized descriptively based on individual study findings. Current evidence in RV clinical trials is outdated and lacks diagnostic and therapeutic standardization. There is a particular need for RV-specific definitions, validated disease activity and response indices, and contemporary therapeutic trials to guide clinical management and improve outcome comparability.

#4

Work-up in patients with retinal vasculitis: A systematic review and meta-analysis: International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) Report 2.

Survey of ophthalmology2026

This systematic review and meta-analysis investigate global diagnostic approaches for retinal vasculitis (RV), with a focus on infectious and non-infectious causes. As in our prior report, systematic searches were conducted in 5 databases and registered in PROSPERO (CRD42023489232). Studies included articles on at least 10 patients with RV without publication date or language restrictions. RV was categorized as secondary RV if they were associated with systemic disease, syndromic if they were associated with a syndromic ocular disease without systemic disease, idiopathic RV without association to systemic or syndromic ocular diseases. This report includes 84 studies analyzing 3480 patients with RV. Among infectious causes, tuberculosis tests were frequently reported, with TST/IGRA positivity observed in 31.4 % (95 % CI: 17.2-50.2 %) of de novo RV cases, increasing to 64.7 % (95 % CI: 47.8-78.9 %) in confirmed tubercular RV and 65.7 % (95 % CI: 39.0-85.1 %) in Eales disease. Chest radiograph abnormalities were present in 21.8 % (95 % CI: 12.9-33.8 %) of tubercular RV cases. Non-infectious causes showed notable regional variability. HLA-B51 positivity was 1 % (95 % CI: 0.03-3.1 %) in de novo RV, but rose to 61.4 % (95 % CI: 23.1-89.4 %) in Behçet's RV. Sarcoidosis RV revealed noncaseating granulomas in 80.5 % (95 % CI: 9.7-99.4 %), with angiotensin-converting enzyme elevation in 4.6 % (95 % CI: 2.3-9.1 %). This study highlights significant geographic variability in RV etiologies. Tuberculosis remains a key infectious cause, while non-infectious causes vary regionally. Region-specific diagnostics and Bayesian testing protocols are critical to improving diagnostic accuracy and patient outcomes.

#5

Focal Choroidal Excavation Associated With Eales Disease.

Retina (Philadelphia, Pa.)2026 Mar 01

Publicações recentes

Ver todas no PubMed

📚 EuropePMC248 artigos no totalmostrando 67

2026

Bilateral Eales' Disease Managed With Vitrectomy and Anti-VEGF Therapy: A Case Report.

The American journal of case reports
2026

Focal Choroidal Excavation Associated With Eales Disease.

Retina (Philadelphia, Pa.)
2026

Combination of Methotrexate and Mycophenolate mofetil for Refractory Uveitis & Scleritis: A Case Series.

Retinal cases &amp; brief reports
2025

Evaluation of changes in mean choroidal thickness before and after treatment with steroids and laser photocoagulation in patients with Eales' disease.

International journal of retina and vitreous
2026

Clinical trials and quasi-experimental studies in the treatment of noninfectious retinal vasculitis: A systematic review from the International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) - Report 4.

Survey of ophthalmology
2026

Work-up in patients with retinal vasculitis: A systematic review and meta-analysis: International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) Report 2.

Survey of ophthalmology
2025

Retinal Vasculitis in the Setting of Mycobacterium tuberculosis Exposure: Clinical Course and Management of Three Cases.

Case reports in ophthalmology
2025

Eales' Disease in Inuit: A Short Report and Clinical Update.

International medical case reports journal
2025

12-year cumulative incidence rate of rare retinal diseases: a nationwide study in Korea.

Eye (London, England)
2024

Eales' disease with secondary coats'-like reaction: a case report.

BMC ophthalmology
2024

A Review of Eales' Disease and Mycobacterium tuberculosis.

Biology
2024

A Multidisciplinary Approach to the Management of Eales Disease: A Case Report and Review of the Literature.

Journal of personalized medicine
2023

Double Trouble: Eales Disease in a Background of Paradoxical Embolism.

Cureus
2023

[Less Frequent Vascular Disorders of the Retina - Part 1].

Klinische Monatsblatter fur Augenheilkunde
2023

Rare case report: a 26-year-old man with Eales' disease.

Romanian journal of ophthalmology
2023

Adjuvant Intravitreal Bevacizumab for Retinal Neovascularization in Eales' Disease Associated With Latent Mycobacterium Tuberculosis.

Cureus
2023

Central retinal artery occlusion as a presenting symptom in Eales' disease: a case report.

Journal of medical case reports
2024

Clinical Features, Long-Term Outcomes, and Prognostic Factors of Eales' Disease in Korean Patients.

Ocular immunology and inflammation
2023

Cocaine Abuse as an Immunological Trigger in a Case Diagnosed with Eales Disease.

Medicina (Kaunas, Lithuania)
2022

Oxidative Stress Implication in Retinal Diseases-A Review.

Antioxidants (Basel, Switzerland)
2022

CLINICAL CHARACTERISTICS OF EALES DISEASE AND THE EFFICACY OF DIFFERENT INTERVENTIONS FOR NEOVASCULAR COMPLICATIONS.

Retina (Philadelphia, Pa.)
2024

A CASE OF EALES DISEASE OBSERVED BY WIDEFIELD OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

Retinal cases &amp; brief reports
2022

Optical coherence tomography angiography features of macular epiretinal neovascularization in Eales' disease: a case report.

Journal of ophthalmic inflammation and infection
2022

Quantile-Dependent Expressivity of Serum Interleukin-6 Concentrations as a Possible Explanation of Gene-Disease Interactions, Gene-Environment Interactions, and Pharmacogenetic Effects.

Inflammation
2022

Eales' disease: epidemiology, diagnostic and therapeutic concepts.

International journal of retina and vitreous
2021

Advanced Eales' Disease With Neovascular Glaucoma at First Presentation.

Cureus
2021

Histopathological, immunohistochemical and molecular biologic study of an enucleated specimen of a case of Eales' disease.

Journal of ophthalmic inflammation and infection
2021

Eales' Disease: When the Rare Sounds Frequent.

Case reports in ophthalmological medicine
2021

Vitrectomy Due to Vitreous Hemorrhage and Tractional Retinal Detachment Secondary to Eales' Disease.

Turkish journal of ophthalmology
2021

Intravitreal Aflibercept as a Rescue Therapy for Retinal Neovascularization and Macular Edema due to Eales Disease.

Case reports in ophthalmological medicine
2020

Papillophlebitis as an initial presentation of Eales' disease.

Oman journal of ophthalmology
2020

Comparison of fundus fluorescein angiography, optical coherence tomography and optical coherence tomography angiography features of macular changes in Eales disease: a case series.

Journal of ophthalmic inflammation and infection
2020

Young Male with Paraparesis and Vision Loss- A Rare Presentation of Eales' Disease.

Annals of Indian Academy of Neurology
2021

Further evidence of the association of latent Mycobacterium tuberculosis in Eales' disease.

International ophthalmology
2020

Ultrasound findings in a case of Eales' disease and ocular trauma with anterior chamber cholesterolosis.

BMC ophthalmology
2020

Role of Ultra-widefield Imaging in Eales' Disease: A Case Series.

Ocular immunology and inflammation
2021

Diagnostic and Therapeutic Challenge.

Retina (Philadelphia, Pa.)
2020

[Treatment of ocular tuberculosis-an update].

Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
2019

Diagnosis of Eales disease from a macular epiretinal membrane.

Archivos de la Sociedad Espanola de Oftalmologia
2019

Wide-field angiography for diagnosis and follow-up of Eales disease: a case series.

Arquivos brasileiros de oftalmologia
2020

Choroidal Tuberculoma Manifesting in A Patient of Eales Disease 6 Years after Initial Presentation.

Ocular immunology and inflammation
2018

Progression of Eales' disease post-partum and long-term follow-up: a case report.

Journal of medical case reports
2018

Ultra-widefield angiography in the diagnosis and management of uveitis.

Taiwan journal of ophthalmology
2018

Role of Intravitreal Bevacizumab in Management of Eale's Disease.

Pakistan journal of medical sciences
2018

Pediatric Eales Disease: An Indian Tertiary Eye Center Experience.

Journal of pediatric ophthalmology and strabismus
2018

Ocular Involvement in Sarcoidosis.

The Journal of rheumatology
2018

Outcomes of transconjunctival sutureless 27-gauge vitrectomy for vitreoretinal diseases.

International journal of ophthalmology
2018

Retinal vasculitis and Eales disease.

Medicina clinica
2017

Eales disease in a young adult man Case report.

Romanian journal of ophthalmology
2018

Spectral domain optical coherence tomography evaluation of macular changes in Eales disease.

Indian journal of ophthalmology
2017

Unilateral Eales' disease a case report.

Romanian journal of ophthalmology
2017

A rare presentation of exudative macroaneurysms in unilateral Eales' disease.

Nepalese journal of ophthalmology : a biannual peer-reviewed academic journal of the Nepal Ophthalmic Society : NEPJOPH
2018

Long-Term Outcomes of a Large Cohort of Patients with Eales' Disease.

Ocular immunology and inflammation
2017

Role of high-resolution computerized tomography chest in identifying tubercular etiology in patients diagnosed as Eales' disease.

Journal of ophthalmic inflammation and infection
2017

The 88-kDa Eales' protein in serum is a complex of haptoglobin, complement C3, and galectin-1 as identified by liquid chromatography coupled mass spectrometry.

Proteomics. Clinical applications
2016

Management of postvitrectomy retinal detachment due to multiple laser-induced retinal holes.

BMJ case reports
2016

Ultra-wide field angiography in the management of Eales disease.

Indian journal of ophthalmology
2016

Nd:YAG laser hyaloidotomy in the management of Premacular Subhyaloid Hemorrhage.

BMC ophthalmology
2017

Steroid-responsive painful ophthalmoplegia: Tolosa-Hunt syndrome, Eales disease, or both?

Cephalalgia : an international journal of headache
2016

[Eales disease and tuberculosis: A case report].

Journal francais d'ophtalmologie
2016

Localization of Human Copper Transporter 1 in the Eye and its Role in Eales Disease.

Ocular immunology and inflammation
2016

Man With Blurry Vision and Eye Pain.

JAMA ophthalmology
2016

Detection of Mycobacterium tuberculosis with nested polymerase chain reaction analysis in enucleated eye ball in Eales' disease.

International ophthalmology
2015

Progressive Nodular Histiocytosis Associated with Eale's Disease.

Indian journal of dermatology
2016

A New Insight of Herbal Promises Against Ocular Disorders: An Occuloinformatics Approach.

Current topics in medicinal chemistry
2015

Interleukin-1β Level Is Increased in Vitreous of Patients with Neovascular Age-Related Macular Degeneration (nAMD) and Polypoidal Choroidal Vasculopathy (PCV).

PloS one
2015

Association of Mycobacterium tuberculosis in the causation of Eales' disease: an institutional experience.

Indian journal of medical microbiology
Ver todos os 248 no EuropePMC

Associações

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Comunidades

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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. Bilateral Eales' Disease Managed With Vitrectomy and Anti-VEGF Therapy: A Case Report.
    The American journal of case reports· 2026· PMID 41785187mais citado
  2. Combination of Methotrexate and Mycophenolate mofetil for Refractory Uveitis &amp; Scleritis: A Case Series.
    Retinal cases &amp; brief reports· 2026· PMID 41687078mais citado
  3. Clinical trials and quasi-experimental studies in the treatment of noninfectious retinal vasculitis: A systematic review from the International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) - Report 4.
    Survey of ophthalmology· 2026· PMID 40983164mais citado
  4. Work-up in patients with retinal vasculitis: A systematic review and meta-analysis: International Uveitis Study Group (IUSG) Retinal Vasculitis Study (ReViSe) Report 2.
    Survey of ophthalmology· 2026· PMID 40972796mais citado
  5. Focal Choroidal Excavation Associated With Eales Disease.
    Retina (Philadelphia, Pa.)· 2026· PMID 41701339mais citado
  6. Atypical Presentation of Eales Disease in a Young Woman.
    Retin Cases Brief Rep· 2026· PMID 41893867recente
  7. Evaluation of changes in mean choroidal thickness before and after treatment with steroids and laser photocoagulation in patients with Eales' disease.
    Int J Retina Vitreous· 2025· PMID 41146356recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:40923(Orphanet)
  2. MONDO:0018460(MONDO)
  3. GARD:6309(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q3772316(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

Doença de Eales
Compêndio · Raras BR

Doença de Eales

ORPHA:40923 · MONDO:0018460
Prevalência
Unknown
Herança
Multigenic/multifactorial, Not applicable
CID-10
H35.0 · Retinopatias de fundo e alterações vasculares da retina
CID-11
Início
Adolescent, Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0271073
EuropePMC
Wikidata
Wikipedia
Papers 10a
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