The Role of Symptom Duration and Serologic Factors in the Relapse of IgG4-Related Ophthalmic Disease following Surgery: A Retrospective Cohort Study.

IgG4-related disease (IgG4-RD) affects multiple organs and is characterized by immune-mediated inflammation and fibrosis; IgG-RD affecting orbital tissue is known as IgG4-related ophthalmic disease (IgG4-ROD). This research is aimed at exploring whether symptom duration and common serologic factors, such as IgG, IgE, and eosinophils, are potential risk factors for IgG4-ROD patient relapse after surgery and identifying possible causes of the positive correlation between symptom duration and relapse. This retrospective cohort study included 40 IgG4-ROD patients after surgery. Auxiliary inspection results were obtained before surgery and during follow-up, and relapse risk factors were identified based on previous studies. We used the Spearman rank correlation test to reveal the relationship between symptom duration and relapse time and identified the optimal cutoff value for symptom duration by X-tile. Then, we divided the patients into the long-duration and short-duration groups. Kaplan-Meier survival analyses and log-rank tests were performed to identify the relationship between symptom duration and relapse using X-tile software. Finally, we studied the relationship between previously studied relapse risk factors and symptom duration. The survival curves of the long-duration and short-duration groups were obviously different, and the baseline serum IgG, IgE, and eosinophil levels and asthma concomitant rate were significantly different between the long-duration and short-duration groups. Furthermore, the baseline serum IgG (r = 0.485, P = 0.002), IgE (r = 0.350, P = 0.037), and eosinophil (r = 0.6535, P < 0.0001) levels were positively correlated with symptom duration. Our study shows that IgG4-ROD symptom duration is significantly positively correlated with relapse rate and negatively correlated with relapse time. Symptom duration was positively correlated with serum baseline IgG4, IgE, and eosinophil levels and asthma history, which were potential risk factors for disease relapse. We recommended that IgG4-ROD patients with symptom durations greater than 96 months continue to receive maintenance steroid therapy longer than 1 year postsurgery to reduce the relapse rate.

Circulating exosomes in ophthalmic disease: novel carriers of biological information circulating exosomes in ophthalmic disease.

Exosomes, small membrane vesicles with a diameter of 30-100 nm, transport lipids, proteins, DNA, and RNA. Exosomes originate from endocytic vessels and are processed and released through exocytosis. They can be taken up by target cells and mediate intercellular communication. Initially, exosomes were thought to be waste products excreted by cells. However, with more research, they have been found to play important roles in physiological and pathological processes. Therefore, they are promising biomarkers for the diagnosis and treatment of a variety of disease conditions, including fundus diseases, ocular surface diseases, retinal diseases, tumors, ocular trauma, and light damage. In this review, we discuss the history, biogenesis, release, isolation, characterization, and biological functions of exosomes, as well as their future application prospects in ophthalmic diseases.

Metformin attenuated histopathological ocular deteriorations in a streptozotocin-induced hyperglycemic rat model.

Diabetes mellitus (DM) often causes ocular disorders leading to vision loss. Metformin is commonly prescribed for type 2 diabetes. This study assessed the effect of metformin on hyperglycemic histopathological eye abnormalities and some possible pathways involved. Male rats were divided into 3 groups (N = 6), namely, healthy control, hyperglycemic non-treated control, and hyperglycemic rats treated with 200 mg/kg metformin. Two weeks after diabetes induction by an intraperitoneal streptozotocin (60 mg streptozotocin (STZ)/kg) injection, the rats develop ocular abnormalities, and metformin (200 mg/kg) treatment was administered daily. Rats underwent dilated retinal digital ophthalmoscope examination and graded for diabetic retinopathy. Rats were sacrificed at 12 weeks, and the cornea, lens, sclera, ciliary body, iris, conjunctiva, retinal, and optic nerve were examined histologically. Rats' fasting blood glucose and body weight were monitored. Serum tumor necrosis factor-α (TNF-α), vascular endothelial growth factor (VEGF), claudin-1, and glutathione/malondialdehyde ratios were analyzed. Metformin significantly attenuated diabetes-related histopathological ocular deteriorations in the cornea, lens, sclera, ciliary body, iris, conjunctiva, retina, and optic nerve partly by restoring serum TNF-α, VEGF, claudin-1, and glutathione/malondialdehyde ratios without significantly affecting the fasting blood glucose levels or body weight in these hyperglycemic rats. Metformin attenuated hyperglycemia-associated histopathological eye deteriorations, possibly partly by ameliorating vascular leakage, oxidative stress, inflammation, and neovascularization, without affecting the fasting blood glucose levels or body weights in these STZ-induced diabetic rats.

Serum albumin and total protein concentration in the tear film of horses with healthy or diseased eyes.

OBJECTIVE: To determine total protein content (TPC) and serum albumin levels in the tears of horses with healthy or diseased eyes. ANIMALS STUDIED: Forty-two horses with healthy eyes and 11 horses with unilateral (n = 10) or bilateral (n = 1) ocular disease. PROCEDURE: Each eye underwent an ophthalmic examination including detailed conjunctivitis scoring and tear collection with Schirmer strips. TPC and serum albumin levels were quantified in tear samples and compared among healthy eyes, affected eyes, and contralateral unaffected eyes. The impact of the following variables on lacrimal protein levels were assessed: age, breed, and sex (healthy eyes), as well as conjunctivitis score (diseased eyes). RESULTS: Lacrimal TPC ranged from 7.0 to 19.5 mg/mL in healthy eyes, while serum albumin ranged from 71.1 to 711.3 µg/mL (~1.6% of TPC) and was higher in tears of aged and female horses (P ≤ .033). Eyes with ocular disease had significantly greater (P ≤ .001) serum albumin in tears (median 679.6 µg/mL) compared to contralateral unaffected eyes (130.0 µg/mL) and eyes of the reference population (200.7 µg/mL). However, lacrimal TPC did not differ significantly among the 3 groups. Scoring of palpebral conjunctival hyperemia trended toward a positive association with serum albumin in tears (r = 0.49, P = .062). CONCLUSIONS: The protein profile in equine tears differs in health and disease. Serum albumin in tears increases with ocular disease and, similar to other species, might serve as a biomarker for ocular insult in horses. Future studies could investigate the protein levels in horses with specific ocular conditions and help determine the biological importance of albumin on the equine ocular surface.

Circular RNAs: Novel Promising Biomarkers in Ocular Diseases.

Circular RNAs (circRNAs) are a novel class of endogenous non-coding RNAs produced by back-splicing. They are found to be expressed in eukaryotic cells and play certain roles in various cellular functions, including fibrosis, cell proliferation, differentiation, apoptosis and angiogenesis. Dysregulated circRNAs are found in several human disorders including, malignancy, vascular, inflammatory as well as nervous diseases. Although, increasing evidence suggests that circRNAs may also contribute in different ocular diseases, the outline of circRNAs in ocular diseases remains obscure. In this review we consider the current state of knowledge regarding the potential role and underlying mechanism of circRNAs in ocular diseases including pterygium, age-related cataract, glaucoma, diabetic retinopathy, retinoblastoma, retinal vascular dysfunction and hyperhomocysteinemia induced ocular diseases, emphasizing that circRNAs could be promising biomarkers for the diagnosis and prognosis evaluation. Future circRNAs-targeted intervention may become a novel therapeutic tool for the treatment of ocular diseases.

Folic acid deficiency and vision: a review.

Folic acid (FA), also termed folate, is an essential vitamin for health at all ages since it participates in the biosynthesis of nucleotides, amino acids, neurotransmitters, and certain vitamins. It is therefore crucial for rapidly growing tissues such as those of the fetus. It is becoming clear that FA deficiency and impaired folate pathways are implicated in many diseases of both early life and old age. FA can be transported into the cell by the folate receptor, the reduced folate transporter, and proton-coupled folate transporter. Folate transport proteins are present in certain eye tissues, which explains why FA plays an important role in eye development. The purpose of this literature review is to investigate the evidence relating FA deficiency to eye diseases.

Early low-dose rituximab for active thyroid eye disease: An effective and well-tolerated treatment.

BACKGROUND: Thyroid eye disease (TED) is an autoimmune inflammatory disease that can be disfiguring and potentially sight threatening. Suppression of inflammation in active disease can reduce the risk of visual loss and limit long-term sequelae. Current management involves inflammation suppression using glucocorticoids. The aim of this study was to evaluate the efficacy of early disease intervention with targeted immunomodulatory therapy to alter disease course. This paper reports the efficacy of low-dose rituximab in reducing clinical activity in TED in a small population. METHODS: A retrospective audit of consecutive patients with active TED managed primarily with a 100 mg rituximab infusion. Further glucocorticoid or steroid-sparing agents were prescribed if clinically indicated. Clinical activity score, VISA overall severity score and Oxford Quality of Life score were recorded at each visit as well as TSH receptor antibody levels (TRAb), B cell subsets and adverse reactions. RESULTS: Twelve patients had mean follow-up of 6.3 months. Clinical activity scores significantly decreased (mean score 5.08 to 1.58; P < 0.001), VISA overall severity scores reduced by 50% from 12 to 6, P < 0.001 and the mean cumulative dose of IV methylprednisolone was 2.3 g. 100 mg rituximab induced significant CD19+ B cell depletion (n = 8, P < 0.001). There was no significant reduction in serum TRAb (n = 8, P = 0.06). A transient infusion-related rash was the only adverse effect, n = 4. QoL scores did not differ markedly before and after treatment. CONCLUSION: Low-dose rituximab is an efficacious, well-tolerated and safe treatment for active TED; reducing disease activity and allowing reduced administration of systemic steroid.

The role of vitamin D in the pathogenesis of ocular diseases.

The prevalence of vitamin D deficiency in the American and European population is estimated to be extremely high. Although fewer people today su er from serious health problems related to calcium and phosphate metabolism resulting from vitamin D deficiency, there are more and more studies suggesting that calcitriol may play an important role in the pathogenesis of other diseases in virtually every body system. A growing body of research shows that through its ubiquitously expressed receptor, calcitriol displays potent anti-angiogenic an anti-inflammatory activity. This review summarizes recent discoveries regarding these non-classical effects of vitamin D and their clinical implications. Data collection focused on the prevention and treatment of ocular diseases as well as on the underlying mechanisms.

Systemic inflammation and eye diseases. The Beijing Eye Study.

PURPOSE: Systemic inflammation is potentially associated with ocular diseases such as late age-related macular degeneration (AMD). Using the serum concentration of high-sensitive C-reactive protein (hs-CRP) as surrogate of systemic inflammation, we examined potential associations between the serum hs-CRP concentration and the presence and degree of eye diseases. METHODS: The population-based Beijing Eye Study included 3468 Chinese individuals. The study participants underwent a standardized interview and a detailed ophthalmic examination. The serum concentration of hs-CRP was determined. RESULTS: Out of 3468 participants, 2452 (70.7%) individuals (mean age:63.4±9.4 year; range:50-91 years) had hs-CRP measurements (mean:1.96±4.07mg/L). In multivariate analysis, higher serum concentration of hs-CRP was significantly (regression coefficient r: 0.21) associated with a higher level of diabetic retinopathy (P = 0.007; standardized regression coefficient beta:0.06; non-standardized regression coefficient B:1.35; 95% confidence interval (CI):0.37,2.22) and polypoidal choroidal vasculopathy (P = 0.002;beta:0.06;B:6.22;95%CI:2.24,10.2) after adjusting for higher serum concentration of high-density lipoproteins (P<0.001;beta:-0.12;B:-1.31;95%CI:-1.77,-0.85), higher body mass index (P = 0.01;beta:0.06;B:0.06;95%CI:0.01, 0.11), lower level of education (P = 0.04;beta:-0.06;B:-0.22;95%CI:-0.42,-0.02), lower cognitive function score (P = 0.01;beta:-0.07;B:-0.08;95%CI:-0.13,-0.02). If the presences of other ocular diseases were added to the model, the presence of glaucoma (P = 0.99), open-angle glaucoma (P = 0.80), angle-closure glaucoma (P = 0.67), pseudoexfoliation (P = 0.18), nuclear cataract (P = 0.30), cortical cataract (P = 0.15), subcapsular cataract (P = 0.59), retinal vein occlusions (P = 0.33), central serous choroidopathy (P = 0.44), early stage of age-related macular degeneration (AMD) (P = 0.46), intermediate stage of AMD (P = 0.20) and late stage of AMD (P = 0.91) including geographic atrophy (P = 0.60) or neovascular AMD (P = 0.68) were not significantly associated with the serum concentration of hs-CRP. CONCLUSIONS: In Chinese aged 50+ years, higher serum concentration of hs-CRP was significantly associated with a higher level of diabetic retinopathy and higher frequency of polypoidal choroidal vasculopathy. Other major ocular disorders, namely glaucoma including open-angle glaucoma and angle-closure glaucoma, pseudoexfoliation, nuclear, cortical or subcapsular cataract, retinal vein occlusions, central serous choroidopathy, early, intermediate or late stage of AMD including geographic atrophy, were not significantly associated with hs-CRP serum concentrations. It suggests that these diseases, in contrast to diabetic retinopathy and polypoidal choroidal vasculopathy, were not associated with a major systemic inflammatory component.

Effect of Repeated Freeze-Thaw on Serum Biomarkers Associated with Eye Disease.

BACKGROUND Serum biomarkers are associated with eye diseases, which results in the need for cryopreservation of serum samples. However, the effect on serum biomarker levels of repeatedly freezing and thawing remains poorly understood. The aim of this study was to evaluate the effects of repeated freeze-thaw on the serum levels of the protein, complement C3c (C3c), the micromolecule, uric acid (UA), and the enzyme, angiotensin-converting enzyme (ACE). MATERIAL AND METHODS Serum samples were obtained from 50 patients who attended an ophthalmic outpatient department. Following baseline measurements, the serum samples from each subject were divided into aliquots and stored at -80°C for further analysis, following between one to six freeze-thaw cycles. The serum levels of C3c, UA, and ACE were measured immediately after the stored serum samples were thawed. RESULTS The serum level of C3c was significantly changed after the first freeze-thaw cycle (p<0.05), and a significant alteration in serum ACE levels occurred after the third freeze-thaw cycle (p<0.05). The serum UA level remained unchanged after all freeze-thaw cycles. Repeated freeze-thaw cycles significantly increased the serum levels of C3c and decreased the serum levels of ACE. The serum levels of C3c, UA, and ACE, respectively were significantly correlated (p<0.001), while the correlation coefficient for C3c and UA were improved when compared with ACE. CONCLUSIONS Repeated freeze-thaw can have variable effects on the serum levels of biomarkers, C3c, UA and ACE, which supports the need for quality control of cryopreserved serum for biomarker evaluation.

Maternal Inheritance of a Recessive RBP4 Defect in Canine Congenital Eye Disease.

Maternally skewed transmission of traits has been associated with genomic imprinting and oocyte-derived mRNA. We report canine congenital eye malformations, caused by an amino acid deletion (K12del) near the N terminus of retinol-binding protein (RBP4). The disease is only expressed when both dam and offspring are deletion homozygotes. RBP carries vitamin A (retinol) from hepatic stores to peripheral tissues, including the placenta and developing eye, where it is required to synthesize retinoic acid. Gestational vitamin A deficiency is a known risk factor for ocular birth defects. The K12del mutation disrupts RBP folding in vivo, decreasing its secretion from hepatocytes to serum. The maternal penetrance effect arises from an impairment in the sequential transfer of retinol across the placenta, via RBP encoded by maternal and fetal genomes. Our results demonstrate a mode of recessive maternal inheritance, with a physiological basis, and they extend previous observations on dominant-negative RBP4 alleles in humans.

Serum Trace Elements Profile in Graves' Disease Patients with or without Orbitopathy in Northeast China.

OBJECTIVE: The purpose of the present study was to investigate serum trace elements in Graves' disease (GD) patients with or without orbitopathy in Northeast China. METHODS: Patients with newly diagnosed Graves' disease (HyGD) (n = 66), GD patients with euthyroid status or subclinical thyroidism after treatment (EUGD) (n = 55), GO patients with euthyroid status or subclinical thyroidism after treatment (GO) (n = 57), and normal controls (NC) (n = 66) were enrolled in this study. Serum trace elements were measured with ICP-MS. RESULTS: Serum selenium (Se) levels in EUGD group (median: 7.53 µg/dL), HyGD group (median: 6.76 µg/dL), and GO group (median: 7.40 µg/dL) were significantly lower than those in NC group (median: 9.20 µg/dL, all P < 0.01). Serum copper (Cu) levels in GO group (median: 95.93 µg/dL) were significantly lower than those in the NC group (median: 113.59 µg/dL, P = 0.015). After being adjusted for multivariables, thyroid-specific antibodies grade was associated with low Se levels. Hyperthyroidism and thyroid-specific antibodies grade were associated with high Cu levels. In addition, orbitopathy was associated with low Cu levels. CONCLUSIONS: Thyroid autoimmunity was associated with low Se levels. Hyperthyroidism and thyroid autoimmunity may be associated with relatively high serum Cu levels. Alternatively, ophthalmopathy may be related to low serum Cu levels.

The role of patient's systemic characteristics and plateletcrit in developing toxic anterior segment syndrome after uneventful phaco surgery: A case-control study.

PURPOSE: To compare the systemic and ocular characteristics and laboratory findings of patients developing toxic anterior segment syndrome (TASS) after uneventful phaco surgery with unaffected subjects undergoing the same surgery in the same session. DESIGN: A retrospective case-control study. METHODS: The study group consisted of 26 eyes of 26 patients who underwent uneventful phaco surgery and who went on to develop TASS, while the control group included 39 subjects who had routine phaco surgery in the same session by the same surgeon. The sterilization stages of reusable instruments, disposable instruments, and compositions were recorded. The preoperative systemic diseases, complete blood count parameters, glycosylated hemoglobin (HbA1c), biochemical parameters, thyroid hormone profiles, and the surgical features were compared between the two groups. RESULTS: Type 2 diabetes mellitus (DM), systemic hypertension (HT), hyperlipidemia, chronic ischaemic heart disease, and chronic renal failure were significantly more common in the TASS group (p < 0.05). Proliferative diabetic retinopathy was also more frequent in the TASS group (p = 0.003). Mean HbA1c% values, white blood cell count, neutrophil/lymphocyte ratio, platelet counts, platelet distribution width, and plateletcrit parameters were significantly higher in the TASS group (p < 0.05). Multivariate logistic regression analysis revealed that a high plateletcrit level (p = 0.001, odds ratio [95% CI]; 22.27 [3.36-147.76]) and systemic HT (p = 0.044, odds ratio [95% CI]; 7.13 [1.05-48.12]) are independently associated with the development of TASS. CONCLUSION: Although TASS may arise as a result of insufficient sterilization of instruments or intraocular solutions, patient factors may also contribute to its development. Systemic vascular disorders such as uncontrolled type 2 DM, systemic hypertension, and hyperlipidemia may increase the risk of TASS after uneventful phaco surgery. Abnormal parameters associated with systemic inflammation, such as higher plateletcrit level, may facilitate the development of TASS. These findings may be a predicting factor of TASS development for uneventful cataract surgeries.

Prevalence of Laboratory Critical Results in Eye Patients from an Eye Hospital in Southern China.

OBJECTIVES: To investigate the prevalence of laboratory critical results (CRs) and associated risk factors in patients with eye diseases in a tertiary eye hospital. METHODS: Blood samples were collected from both inpatients and outpatients at Zhongshan Ophthalmic Center, Guangzhou, China, from June 1, 2012, to May 31, 2014, and samples were sent to the hospital's clinical laboratory for blood routine, biochemistry, and blood coagulation tests. Laboratory CRs for blood glucose, sodium, potassium, white blood cell count, platelet count, prothrombin time, fibrinogen, international normalized ratio, and activated partial thromboplastin time were included in the current analysis. RESULTS: A total of 60403 subjects were enrolled in the current analysis. CRs were identified in 339 tests from 336 patients with a prevalence of 5.7‰. Age was positively associated with the presence of CRs. Compared to patients with lens diseases, patients with strabismus, oculoplastics, and ocular trauma were less likely to have CRs (P < 0.05), while patients with tumors were more likely to have CRs (P < 0.001). CONCLUSIONS: The prevalence of CRs in eye patients is low but calls for medication attention. It is important for medical personnel, especially ophthalmologists, to increase awareness of the importance, as well as the prevalence and risk factors of CRs.

Plasma rico en plaquetas en superficie ocular / Platelet rich plasma in ocular surface

El uso de los preparados ricos en plaquetas ha experimentado un aumento significativo en los últimos años debido a su papel en la reparación y regeneración tisular. El objetivo del presente estudio es recopilar la evidencia disponible respecto a la aplicación de plasma rico en factores de crecimiento y sus variantes sobre la superficie ocular: el efecto de los factores de crecimiento derivados de plaquetas, las implicaciones de los distintos métodos de preparación, los estudios publicados en patologías de la superficie ocular, así como sus contraindicaciones y reacciones adversas. Pese al uso generalizado de los preparados de plaquetas, no existe un consenso sobre el método de preparación más adecuado, variando las concentraciones de factores de crecimiento según el sistema empleado. Estos preparados se han utilizado en el tratamiento de enfermedades de la superficie ocular como del ojo seco o los defectos epiteliales persistentes, entre otras, con un perfil adecuado de eficacia y seguridad, aunque son necesarios más estudios para su posicionamiento terapéutico respecto a las alternativas actualmente disponibles

The use of platelet-rich preparations has experienced a significant increase in recent years due to its role in tissue-repair and regeneration. The aim of this study is to examine the available evidence regarding the application of plasma rich in growth factors, and its variations, on the ocular surface. A review is also presented on the effects of platelet-derived growth factors, the implications of the preparation methods, and the existing literature on the safety and efficacy of these therapies in ocular surface diseases. Despite the widespread use of platelet preparations there is no consensus on the most appropriate preparation method, and growth factors concentration vary with different systems. These preparations have been used in the treatment of ocular surface diseases, such as dry eye or persistent epithelial defects, among others, with good safety and efficacy profiles, but further studies are needed to compare to the currently available alternatives

Beyond Joints: a Review of Ocular Abnormalities in Gout and Hyperuricemia.

Gout is a common inflammatory arthritis among middle-aged men and postmenopausal women and can be a debilitating disease. Gout results from an elevated body uric acid pool, which leads to deposition of monosodium urate (MSU) crystals, mainly in and around the joints. The MSU crystals trigger release of proinflammatory cytokines, such as interleukin (IL)-1ß, IL-6, and tumor necrosis factor (TNF)-α. Ocular manifestations have been uncommonly reported in patients with gout. These include descriptions of tophaceous deposits in different locations of the eye including the eyelids, conjunctiva, cornea, iris, sclera, and orbit. Some depositions were coincidentally diagnosed in asymptomatic patients, while the majority were symptomatic. Other ocular abnormalities include dry eye syndrome, red eye, uveitis, intraocular hypertension, glaucoma, and cataracts. Herein, we review the medical literature pertaining to ocular manifestations in gout and hyperuricemia and propose a possible association between ocular abnormalities, hyperuricemia, and gout, including their common risk factors and comorbidities.

[A clinicopathological study of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular disorders].

OBJECTIVE: To study the clinicopathologic features of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular disorders. METHODS: Forty-three cases were selected from the Department of Pathology, West China Hospital, Sichuan University from December 2008 to January 2015 with retrospective series case study. The clinical manifestation, laboratory examination, imaging findings, treatment and histopathological characteristics were analyzed. According to the diagnostic criteria for IgG4-related ocular disease in 2014 and relevant literatures, our cases were divided into two groups: IgG4-related ocular disease (23 samples from 18patients) and non-IgG4-related ocular disease (31 samples from 25 patients). The male-to-female ratio, the periductal fibrosis and the orbital lesion area was analyzed by Pearson chi-square. The Immunohistochemistry of IgG4+/HPF, IgG+/HPF and IgG4+/IgG+ ratio and age was analyzed by two sample t test. The involved structural tissue and the infiltration of eosinophils and lymphocytes and plasma cells was analyzed by signed-rank test. RESULTS: The significant statistic differences were observed between these two groups in immunohistochemical staining of IgG4 (>40/HPF) (t=12.17, P<0.01), IgG (t=2.91, P<0.01) and IgG4+/IgG+ ratio (≥40%) (t=16.98, P<0.01), the lymphocytic and plasmacytic infiltration (F=3.62, P=0.03),the periductal fibrosis(F=4.71 , P=0.03), the involved region of orbit (t=1.11, P=0.01), but not in the mean age and male-to-female ratio (t=1.09, P=0.58). IgG4 ROD should be distinguished form the non-specific inflammation disorders, lymphoma and so on. CONCLUSIONS: IgG4-related ocular disease often arises from middle-old man, characterized by bilateral orbital painless swelling and abundant IgG4+ plasma cells infiltration. Nearly all orbital tissue could be involved by IgG4-related ocular disease, especially lacrimal gland. But the characteristic obliterating phlebitis were not seen. The immunohistochemical staining of IgG4 and IgG plays an important role in differentiating IgG4-related ocular disease from non-IgG4-related ocular disorders. Combining clinical manifestation, laboratory examination, imaging findings with histopathology is helpful for diagnosis and differential diagnosis. (Chin J Ophthalmol, 2016, 52: 256-262).