A síndrome de Posner-Schlossman (SPS), também conhecida como crise glaucomatociclítica (CGC), é uma condição ocular aguda rara com ataques unilaterais de uveíte anterior granulomatosa leve e pressão intraocular elevada. Às vezes, é considerada um glaucoma inflamatório secundário.
Introdução
O que você precisa saber de cara
Visão geral
A doença glaucomatociclítica por crises, também registrada pelo identificador MONDO:0004772 e CID-10 H40.4, tem sua prevalência classificada como desconhecida. O início da condição ocorre tipicamente em adolescentes e adultos.[1]
Causas genéticas
Diagnóstico
Tratamento e manejo
No Sistema Único de Saúde (SUS), o manejo associado ao protocolo de Glaucoma (Portaria Conjunta SAES-SECTICS/MS nº 28, de 06 de dezembro de 2023) conta com medicamentos dispensados pelas farmácias públicas, incluindo opções como Tartarato de Brimonidina (solução oftálmica 2 mg/mL), Travoprosta (solução oftálmica 0,04 mg/mL), Cloridrato de dorzolamida (solução oftálmica 20 mg/mL), Cloridrato de pilocarpina (solução oftálmica 20 mg/mL - 2%), Bimatoprosta (solução oftálmica 0,3 mg/mL - 0,03%), Maleato de timolol (soluções oftálmicas 2,5 mg/mL e 5 mg/mL), Brinzolamida (suspensão oftálmica 10 mg/mL) e Latanoprosta (solução oftálmica 0,05 mg/mL).[3][3][3][3][3][3][3][3][3]
Além das soluções oftálmicas, o SUS disponibiliza para o manejo de glaucoma a Acetazolamida em comprimidos de 250 mg e a solução injetável de Manitol a 20%, conforme a mesma portaria ministerial de dezembro de 2023.[3][3]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
A doença glaucomatociclítica por crises, também chamada de Síndrome Posner-Schlossman, é uma condição ocular rara que causa episódios de elevação na pressão do olho e inflamação leve. Os sintomas costumam iniciar na adolescência ou na fase adulta e requerem acompanhamento periódico pelo risco de lesões no nervo óptico. Embora a doença não tenha um protocolo exclusivo para doenças raras no Ministério da Saúde, o SUS dispensa opções de colírios e medicamentos para controle pressórico por meio do PCDT de Glaucoma.
A síndrome de Posner-Schlossman (SPS), também conhecida como crise glaucomatociclítica (CGC), é uma condição ocular aguda rara com ataques unilaterais de uveíte anterior granulomatosa leve e pressão intraocular elevada. Às vezes, é considerada um glaucoma inflamatório secundário.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
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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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
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Publicações mais relevantes
The Incidence and Risk Factors of Optic Nerve Damage of Posner-Schlossman Syndrome: A Meta-Analysis.
To explore the incidence and associated factors of glaucoma optic nerve damage (GOND) among patients with Posner-Schlossman syndrome. We retrieved relative studies till July 2022 using databases including PubMed, CNKI, VIP, and Wan-Fang. The retrieval terms include "Posner-Schlossman syndrome", "Glaucomatocyclitic Crisis", and "visual field". The Chinese retrieval terms were the corresponding Chinese terminologies for the English terms mentioned above. The outcomes were the incidence of GOND among PSS patients, the male proportion, patient age, the proportion of patients with single eye affected, disease duration, and intraocular pressure during the episode in patients with or without GOND. Review manager 5.3 was used for the analysis. In total, 19 studies were included in our analysis. The pooled incidence of GOND among PSS patients was 0.26 (95% CI = 0.16-0.43). Age [MD = 11.3(5.86, 16.73); P < .0001], disease duration [MD = 4.27 (3.38, 5.16), P < .00001], and single or double eye affected [RR = 0.69 (0.49, 0.98), P = .04] were significantly associated with the development of GOND. Whereas, gender [RR = 1.09 (0.91, 1.29), P = .35] and intraocular pressure at episodes [MD = 2.66 (-0.38, 5.7), P = .09] were not significantly associated with GOND development. A fraction of PSS patients ultimately develop GOND so physicians should not be highly optimistic about the prognosis of PSS patients and timely and effective treatment is very important. Patients of higher age, those with double eyes affected, and suffering from a long disease duration might be at a greater risk of developing GOND.
Acute exposure to air pollutants increase the risk of acute glaucoma.
Ambient air pollution is related to the onset and progression of ocular disease. However, the effect of air pollutants on the acute glaucoma remains unclear. To investigate the effect of air pollutants on the incidence of acute glaucoma (acute angle closure glaucoma and glaucomatocyclitic crisis) among adults. We conducted a time-stratified case-crossover study based on the data of glaucoma outpatients from January, 2015 to Dec, 2021 in Shanghai, China. A conditional logistic regression model combined with a polynomial distributed lag model was applied for the statistical analysis. Each case serves as its own referent by comparing exposures on the day of the outpatient visit to the exposures on the other 3-4 control days on the same week, month and year. To fully capture the delayed effect of air pollution, we used a maximum lag of 7 days in main model. A total of 14,385 acute glaucoma outpatients were included in this study. We found exposure to PM2.5, PM10, nitrogen dioxide (NO2) and carbon monoxide (CO) significantly increased the odds of outpatient visit for acute glaucoma. Wherein the odds of acute glaucoma related to PM2.5 and NO2 were higher and more sustained, with OR of 1.07 (95%CI: 1.03-1.11) and 1.12 (95% CI: 1.08-1.17) for an IQR increase over lag 0-3 days, than PM10 and CO over lag 0-1 days (OR:1.03; 95% CI: 1.01-1.05; OR: 1.04; 95% CI: 1.01-1.07). This case-crossover study provided first-hand evidence that air pollutants, especially PM2.5 and NO2, significantly increased risk of acute glaucoma.
CFH I62V as a Putative Genetic Marker for Posner-Schlossman Syndrome.
Objective: Posner-Schlossman syndrome (PSS), also known as glaucomatocyclitic crisis, is an ocular condition characterized by recurrent attacks of anterior uveitis and raised intraocular pressure. Previous studies by our team and others have identified the genetic association of complement pathway genes with uveitis and glaucoma. This study aimed to investigate the complement genes in PSS patients with the view of elucidating the genetic background of the disease. Methods: A total of 331 subjects (56 PSS patients and 275 controls) were recruited for this study. We selected 27 variants in six complement pathway genes (SERPING1, C2, CFB, CFH, C3, and C5) and detected them using TaqMan single nucleotide polymorphism (SNP) Genotyping Assays. Univariate SNP association analysis, haplotype-based association analysis, gene-gene interaction analysis among complement genes, and genotype-phenotype correlation analysis were performed. Results: Among the 27 variants of six complement pathway genes, the functional variant I62V (rs800292) at the CFH gene was found to be significantly associated with PSS; there was a significant increase in the frequency of A allele and AA homozygosity in PSS patients than in controls (P = 1.79 × 10-4; odds ratio (OR) 2.18, 95% CI: 1.44-3.29; P = 4.65 × 10-4; OR 3.66, 95% CI: 1.70-7.85, respectively). The additive effect of CFH-rs800292 and SERPING1-rs3824988 was identified with an OR of 12.50 (95% CI: 2.16-72.28). Genotype-phenotype analysis indicated that the rs800292 AA genotype was associated with a higher intraocular pressure and higher frequency of recurrence. Unlike a high proportion of human leukocyte antigen (HLA)-B27 positivity in anterior uveitis, only 3 in 56 (5.36%) PSS patients were HLA-B27 positive. In addition, one haplotype block (GC) in the SERPING1 gene showed a nominal association with PSS with an increased risk of 2.04 (P = 0.01; 95% CI: 1.18-3.53), but the P-value could not withstand the Bonferroni correction (Pcorr > 0.05). Conclusion: This study revealed a genetic association of a CFH variant with PSS as well as its clinical parameters, implying that the alternative complement pathway might play an important role in the pathogenesis of PSS. Further studies to enrich the understanding of the genetic background of PSS and the role of the complement system in ocular inflammation are warranted.
Posner-Schlossman Syndrome in Common Variable Immunodeficiency.
Posner-Schlossman syndrome (PSS) is a rare glaucomatocyclitic crisis with clinical features including recurrent episodes of unilateral elevated intraocular pressure. Autoimmune and infectious causes have been proposed as potential etiologies of PSS. We report the first case of PSS in the setting of common variable immunodeficiency (CVID). Case Report. A sixty-two-year-old Caucasian female with a medical history of CVID and ulcerative colitis presented to the emergency room with complaints of acute right-sided vision changes. She reported image distortion, blurriness, and loss of central vision. Physical exam was significant for mildly injected right conjunctiva, visual acuity of 20/70 in right eye, and 20/25 in left eye. The right intraocular pressure was measured at 34 mmHg and left at 12 mmHg. The gonioscopy and dilated fundus examination were unremarkable. Cup to disc ratio was within normal limits, and no afferent pupillary defects were recorded. The patient was acutely treated with three rounds of dorzolamide/timolol and 0.2% brimonidine which decreased the right eye intraocular pressure to 24 mmHg. On follow-up exam with an ophthalmologist, anterior uveitis including an elevated pressure of 41 mmHg on the right and 18 mmHg on the left eye was noted and a PSS diagnosis was confirmed. PSS remains a rare condition with uncertain etiology and no associated systemic conditions. PSS has been postulated to be linked to autoimmune conditions. CVID is associated with many autoimmune disorders including Sjogren's, rheumatoid arthritis, and colitis. There have been a few reported CVID-associated ocular diseases including granulomatous uveitis and conjunctivitis, chronic anterior uveitis, and birdshot retinopathy. We describe the first case of PSS in a patient with CVID.
A meta-analysis of the rate and related factors of glaucomatous optic nerve damage in patients with Glaucomatocyclitic Crisis.
To systematic analysis of domestic and foreign literature on the incidence and related factors of glaucomatous optic nerve damage (GOND) in patients with Glaucomatocyclitic Crisis (PSS). A computerized literature search was carried out in PubMed database, Wanfang Medical Database, China National Knowledge Infrastructure to collected domestic and foreign research studies on the incidence and related factors of glaucomatous optic nerve damage of Glaucomatocyclitic Crisis. Using Stata 15.1 software, select the indicators: incidence, gender, age, single/double eyes, duration of disease, and intraocular pressure (IOP) at onset for meta-analysis. OR (odds risk) was used as the effect variable for the binary variables, and mean difference was used as the effect variable for the continuous variables. The results are expressed by each effect amount and its 95% Confidence interval (CI). If there was heterogeneity among the original studies, a random effects model was used; otherwise, a fixed effects model was used. A total of 13 studies were included. The incidence of GOND in PSS was 0.251 (95%CI: 0.175-0.327). Three studies include the relevant factor analysis and the results showed: there was a statistically significant difference in age, and duration of disease in PSS patients with/without GOND (p = 0.000, p = 0.000), there was no statistical difference between the two groups in gender, single /double eyes, and IOP at onset (p = 0.468, 0.053, 0.065). Glaucomatocyclitic Crisis can cause glaucomatous optic nerve damage. GOND is more likely to occur in patients who are older, and have a long course of the disease. For such patients, special attention should be paid to the detailed examination of visual function and follow-up.
Publicações recentes
Posner-Schlossman Syndrome.
Preliminary research of the clinical pathways of glaucomatous optic nerve damage in cases with glaucomatocyclitic crisis: A retrospective study.
The Use of ChatGPT to Assist in Diagnosing Glaucoma Based on Clinical Case Reports.
The Incidence and Risk Factors of Optic Nerve Damage of Posner-Schlossman Syndrome: A Meta-Analysis.
Acute exposure to air pollutants increase the risk of acute glaucoma.
📚 EuropePMCmostrando 8
The Incidence and Risk Factors of Optic Nerve Damage of Posner-Schlossman Syndrome: A Meta-Analysis.
Alternative therapies in health and medicineAcute exposure to air pollutants increase the risk of acute glaucoma.
BMC public healthCFH I62V as a Putative Genetic Marker for Posner-Schlossman Syndrome.
Frontiers in immunologyPosner-Schlossman Syndrome in Common Variable Immunodeficiency.
Case reports in ophthalmological medicineA meta-analysis of the rate and related factors of glaucomatous optic nerve damage in patients with Glaucomatocyclitic Crisis.
International ophthalmologyThe characteristic of intraocular pressure dynamic change in patients with glaucomatocyclitic crisis.
International ophthalmologyPosner-Schlossman syndrome in Wenzhou, China: a retrospective review study.
The British journal of ophthalmologyPosner-Schlossman syndrome.
Survey of ophthalmologyAssociações
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É uma afecção ocular rara, também chamada de Síndrome Posner-Schlossman, classificada pelo código CID-10 H40.4 e ORPHA:636950. Ela se caracteriza por episódios recorrentes que alteram a dinâmica da pressão intraocular.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
4 fontes citadas no texto · 8 publicações do grafo RarasNet (PubMed) · 2 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Doença glaucomatociclítica por crises. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/doenca-glaucomatociclitica-por-crises
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Doença glaucomatociclítica por crises
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- Reposicionamento
- fonte: Drug Repurposing Hub