Nonspecific Orbital Inflammation Research Articles

Bioinformatic validation and machine learning-based exploration of purine metabolism-related gene signatures in the context of immunotherapeutic strategies for nonspecific orbital inflammation.

Nonspecific orbital inflammation (NSOI) represents a perplexing and persistent proliferative inflammatory disorder of idiopathic nature, characterized by a heterogeneous lymphoid infiltration within the orbital region. This condition, marked by the aberrant metabolic activities of its cellular constituents, starkly contrasts with the metabolic equilibrium found in healthy cells. Among the myriad pathways integral to cellular metabolism, purine metabolism emerges as a critical player, providing the building blocks for nucleic acid synthesis, such as DNA and RNA. Despite its significance, the contribution of Purine Metabolism Genes (PMGs) to the pathophysiological landscape of NSOI remains a mystery, highlighting a critical gap in our understanding of the disease's molecular underpinnings. To bridge this knowledge gap, our study embarked on an exploratory journey to identify and validate PMGs implicated in NSOI, employing a comprehensive bioinformatics strategy. By intersecting differential gene expression analyses with a curated list of 92 known PMGs, we aimed to pinpoint those with potential roles in NSOI. Advanced methodologies, including Gene Set Enrichment Analysis (GSEA) and Gene Set Variation Analysis (GSVA), facilitated a deep dive into the biological functions and pathways associated with these PMGs. Further refinement through Lasso regression and Support Vector Machine-Recursive Feature Elimination (SVM-RFE) enabled the identification of key hub genes and the evaluation of their diagnostic prowess for NSOI. Additionally, the relationship between these hub PMGs and relevant clinical parameters was thoroughly investigated. To corroborate our findings, we analyzed expression data from datasets GSE58331 and GSE105149, focusing on the seven PMGs identified as potentially crucial to NSOI pathology. Our investigation unveiled seven PMGs (ENTPD1, POLR2K, NPR2, PDE6D, PDE6H, PDE4B, and ALLC) as intimately connected to NSOI. Functional analyses shed light on their involvement in processes such as peroxisome targeting sequence binding, seminiferous tubule development, and ciliary transition zone organization. Importantly, the diagnostic capabilities of these PMGs demonstrated promising efficacy in distinguishing NSOI from non-affected states. Through rigorous bioinformatics analyses, this study unveils seven PMGs as novel biomarker candidates for NSOI, elucidating their potential roles in the disease's pathogenesis. These discoveries not only enhance our understanding of NSOI at the molecular level but also pave the way for innovative approaches to monitor and study its progression, offering a beacon of hope for individuals afflicted by this enigmatic condition.

GLAUCOMA SECONDARY TO NONSPECIFIC ORBITAL INFLAMMATION: A DIAGNOSIS NOT TO OVERLOOK

Introduction : Nonspecific Orbital Inflammation (NSOI) is an inflammatory condition of unknown etiology manifesting as pain, ocular motility disturbances, eyelid edema, chemosis, and increased intraocular pressure (IOP). We illustrate a case of glaucoma secondary to NSOI and the importance of understanding the cause-effect relationship between the two conditions.
 Case Illustration : An eighteen-year-old girl presented with severe pain, ptosis, chemosis, ophthalmoplegia, and increased IOP (38 mmHg) despite a leaking trabeculectomy bleb, on the left eye. She was referredwith the diagnosis of primary angle-closure glaucoma (PACG) and a history of twice trabeculectomy surgeries done elsewhere two months before she came to our hospital. She could not move her lefteye nasally five months before the first surgery and felt joint pains for a year. After thorough diagnostics, she was assessed with left eye NSOI and secondary glaucoma with spondyloarthropathy. Oral prednisone 30 mg/day was given to manage the inflammation and the resultant glaucoma. One week follow-up showed an improved condition with decreased IOP (16 mmHg) despite the same anti-glaucoma therapy regimen.
 Discussion : NSOI can mimic various diseases, making diagnosis challenging. The raised IOP in NSOI is often caused by extraocular muscles and orbital tissue swelling, which compresses the superior and inferior ophthalmic veins, resulting in choroidal circulation problems. The IOP was quickly reduced once the inflammation was managed. This is a case of secondary glaucoma caused by an overlooked NSOI, aggravated by the immunological process in the orbit after trabeculectomy surgeries.
 Conclusion : Secondary glaucoma should be considered as a differential diagnosis of PACG in patients with NSOI.

Nonspecific Orbital Inflammation (NSOI): Unraveling the Molecular Pathogenesis, Diagnostic Modalities, and Therapeutic Interventions.

Nonspecific orbital inflammation (NSOI), colloquially known as orbital pseudotumor, sometimes presents a diagnostic and therapeutic challenge in ophthalmology. This review aims to dissect NSOI through a molecular lens, offering a comprehensive overview of its pathogenesis, clinical presentation, diagnostic methods, and management strategies. The article delves into the underpinnings of NSOI, examining immunological and environmental factors alongside intricate molecular mechanisms involving signaling pathways, cytokines, and mediators. Special emphasis is placed on emerging molecular discoveries and approaches, highlighting the significance of understanding molecular mechanisms in NSOI for the development of novel diagnostic and therapeutic tools. Various diagnostic modalities are scrutinized for their utility and limitations. Therapeutic interventions encompass medical treatments with corticosteroids and immunomodulatory agents, all discussed in light of current molecular understanding. More importantly, this review offers a novel molecular perspective on NSOI, dissecting its pathogenesis and management with an emphasis on the latest molecular discoveries. It introduces an integrated approach combining advanced molecular diagnostics with current clinical assessments and explores emerging targeted therapies. By synthesizing these facets, the review aims to inform clinicians and researchers alike, paving the way for molecularly informed, precision-based strategies for managing NSOI.

Glutamine metabolism-related genes and immunotherapy in nonspecific orbital inflammation were validated using bioinformatics and machine learning

BackgroundNonspecific orbital inflammation (NSOI) is an idiopathic, persistent, and proliferative inflammatory condition affecting the orbit, characterized by polymorphous lymphoid infiltration. Its pathogenesis and progression have been linked to imbalances in tumor metabolic pathways, with glutamine (Gln) metabolism emerging as a critical aspect in cancer. Metabolic reprogramming is known to influence clinical outcomes in various malignancies. However, comprehensive research on glutamine metabolism's significance in NSOI is lacking.MethodsThis study conducted a bioinformatics analysis to identify and validate potential glutamine-related molecules (GlnMgs) associated with NSOI. The discovery of GlnMgs involved the intersection of differential expression analysis with a set of 42 candidate GlnMgs. The biological functions and pathways of the identified GlnMgs were analyzed using GSEA and GSVA. Lasso regression and SVM-RFE methods identified hub genes and assessed the diagnostic efficacy of fourteen GlnMgs in NSOI. The correlation between hub GlnMgs and clinical characteristics was also examined. The expression levels of the fourteen GlnMgs were validated using datasets GSE58331 and GSE105149.ResultsFourteen GlnMgs related to NSOI were identified, including FTCD, CPS1, CTPS1, NAGS, DDAH2, PHGDH, GGT1, GCLM, GLUD1, ART4, AADAT, ASNSD1, SLC38A1, and GFPT2. Biological function analysis indicated their involvement in responses to extracellular stimulus, mitochondrial matrix, and lipid transport. The diagnostic performance of these GlnMgs in distinguishing NSOI showed promising results.ConclusionsThis study successfully identified fourteen GlnMgs associated with NSOI, providing insights into potential novel biomarkers for NSOI and avenues for monitoring disease progression.

Inflammation and Fibrosis in Orbital Inflammatory Disease: A Histopathologic Analysis.

The purpose of this study was to compare the histopathologic inflammation and fibrosis of orbital adipose tissue in orbital inflammatory disease (OID) specimens. In this retrospective cohort study, inflammation, and fibrosis in orbital adipose tissue from patients with thyroid-associated orbitopathy (TAO), granulomatosis with polyangiitis (GPA), sarcoidosis, nonspecific orbital inflammation (NSOI), and healthy controls were scored by 2 masked ocular pathologists. Both categories were scored on a scale of 0 to 3 with scoring criteria based on the percentage of specimens containing inflammation or fibrosis, respectively. Tissue specimens were collected from oculoplastic surgeons at 8 international centers representing 4 countries. Seventy-four specimens were included: 25 with TAO, 6 with orbital GPA, 7 with orbital sarcoidosis, 24 with NSOI, and 12 healthy controls. The mean inflammation and fibrosis scores for healthy controls were 0.0 and 1.1, respectively. Orbital inflammatory disease groups' inflammation (I) and fibrosis (F) scores, formatted [I, F] with respective p -values when compared to controls, were: TAO [0.2, 1.4] ( p = 1, 1), GPA [1.9, 2.6] ( p = 0.003, 0.009), sarcoidosis [2.4, 1.9] ( p = 0.001, 0.023), and NSOI [1.3, 1.8] ( p ≤ 0.001, 0.018). Sarcoidosis had the highest mean inflammation score. The pairwise analysis demonstrated that sarcoidosis had a significantly higher mean inflammation score than NSOI ( p = 0.036) and TAO ( p < 0.0001), but no difference when compared to GPA. GPA had the highest mean fibrosis score, with pairwise analysis demonstrating a significantly higher mean fibrosis score than TAO ( p = 0.048). Mean inflammation and fibrosis scores in TAO orbital adipose tissue samples did not differ from healthy controls. In contrast, the more "intense" inflammatory diseases such as GPA, sarcoidosis, and NSOI did demonstrate higher histopathologic inflammation and fibrosis. This has implications in prognosis, therapeutic selection, and response monitoring in orbital inflammatory disease.

Weak acid-initiated slow release of Dexamethasone from hydrogel to treat orbital inflammation.

Rationale: Orbital inflammation is a prevalent and prolonged ocular disease that poses a significant challenge to clinicians. Glucocorticoid Dexamethasone sodium phosphate (Dex) has demonstrated efficacy in the clinical treatment of nonspecific orbital inflammation. However, frequent administration is required due to the short half-life of Dex, which may lead to drug waste and adverse side effects. Methods: In this study, we co-assembled Dex with a weak acid responsive hydrogelator Py-Phe-Phe-Lys-Lys-OH (K) to obtain a novel supramolecular hydrogel Dex/K that could release Dex in a slow manner to treat orbital inflammation. The therapeutic effect of Gel Dex/K on orbital inflammation was verified by in vitro and in vivo experiments. Results: In vitro experiments indicated that co-assembly of Dex with K significantly increased mechanic strength of the hydrogel, enabling a continuous release of 40% of total Dex within 7 days. In vivo experiments further demonstrated that sustained release of Dex from Gel Dex/K could effectively alleviate the infiltration of inflammatory cells and the release of inflammatory factors in the orbit of mice, improving symptoms such as increased intraocular pressure and proptosis. Additionally, Gel Dex/K mitigated the degree of tissue fibrosis and fatty infiltration by reducing the development of local inflammation in the orbit. Conclusions: Our research results indicate that Gel Dex/K could more efficiently achieve responsive drug release in orbit, providing an innovative method for treating orbital inflammation.

Novel Insights into Pathophysiology of Orbital Inflammatory Diseases and Progression to Orbital Lymphoma by Pathway Enrichment Analysis.

Non-specific orbital inflammation (NSOI) and IgG4-related orbital disease (IgG4-ROD) are currently treated with non-specific immunosuppressive agents based on non-randomized, uncontrolled studies. Therefore, relapses and prolongated courses are common and remain challenging. For a more specific therapy, a better understanding of the underlying pathophysiology is crucial. Therefore, we aimed to analyze signaling pathways to expand the knowledge on the pathophysiology and possibly identify specific targets in the future, as occurred recently in Graves’ orbitopathy with the IGF-1 receptor. Furthermore, we analyzed potential mechanisms for the described potential progression to orbital MALT (mucosa-associated lymphoid tissue) lymphoma. The investigation cohort for this screening study comprised of 12 patients with either typical NSOI (n = 6), IgG4-ROD or MALT lymphoma (n = 3 each). Mean age was 56.4 ± 17 years. MALT samples, in contrast with IgG4-ROD and NSOI, showed overall upregulation for extracellular matrix receptor interaction (ECM) and adipocytokine signaling. Investigating signaling compounds for MALT samples, differentially expressed genes were re-identified as targets with relevant expression. Even though pathway analysis showed differentially altered products when comparing IgG4-ROD with MALT, main conductors of differentiation in B- and T-cell signaling were commonly altered when observing the microenvironment of examined tissues. Our data reveal the characteristic differences and similarities in genetic-expression-based pathway profiles between MALT lymphoma, IgG4-ROD and NSOI, which may be useful for elucidating the associated pathogenic mechanisms and developing specific treatments for these orbital diseases.

Evaluation of Orbital Lymphoproliferative and Inflammatory Disorders by Gene Expression Analysis.

Non-specific orbital inflammation (NSOI) and IgG4-related orbital disease (IgG4-ROD) are often challenging to differentiate. Furthermore, it is still uncertain how chronic inflammation, such as IgG4-ROD, can lead to mucosa-associated lymphoid tissue (MALT) lymphoma. Therefore, we aimed to evaluate the diagnostic value of gene expression analysis to differentiate orbital autoimmune diseases and elucidate genetic overlaps. First, we established a database of NSOI, relapsing NSOI, IgG4-ROD and MALT lymphoma patients of our orbital center (2000–2019). In a consensus process, three typical patients of the above mentioned three groups (mean age 56.4 ± 17 years) at similar locations were selected. Afterwards, RNA was isolated using the RNeasy FFPE kit (Qiagen) from archived paraffin-embedded tissues. The RNA of these 12 patients were then subjected to gene expression analysis (NanoString nCounter®), including a total of 1364 target genes. The most significantly upregulated and downregulated genes were used for a machine learning algorithm to distinguish entities. This was possible with a high probability (p < 0.0001). Interestingly, gene expression patterns showed a characteristic overlap of lymphoma with IgG4-ROD and NSOI. In contrast, IgG4-ROD shared only altered expression of one gene regarding NSOI. To validate our potential biomarker genes, we isolated the RNA of a further 48 patients (24 NSOI, 11 IgG4-ROD, 13 lymphoma patients). Then, gene expression pattern analysis of the 35 identified target genes was performed using a custom-designed CodeSet to assess the prediction accuracy of the multi-parameter scoring algorithms. They showed high accuracy and good performance (AUC ROC: IgG4-ROD 0.81, MALT 0.82, NSOI 0.67). To conclude, genetic expression analysis has the potential for faster and more secure differentiation between NSOI and IgG4-ROD. MALT-lymphoma and IgG4-ROD showed more genetic similarities, which points towards progression to lymphoma.

Systemic inflammatory markers differentiate between orbital cellulitis and non-specific orbital inflammation

ABSTRACT Purpose Bacterial orbital cellulitis (OC) and diffuse non-specific orbital inflammation (DNSOI) may be challenging to differentiate clinically. This study investigates the utility of systemic inflammatory markers, namely white cell count (WCC) and C-reactive protein (CRP), in differentiating between OC and DNSOI. Methods A single-centre retrospective study of patients diagnosed with OC or DNSOI, between 2003 to 2021, who had WCC and/or CRP obtained at presentation. The mean levels of these factors and the proportion of positivity were compared between OC and DNSOI. A receiver operating characteristic (ROC) analysis was conducted to calculate the specificity and sensitivity of WCC or CRP in each group. Results 49 patients were included in this study. The mean age was 56 ± 20 years, and 21 patients were females. 26 (53.1%) patients had OC, and 23 (46.9%) patients had DNSOI. Mean WCC for OC and DNSOI were 14.5 × 103/μL and 9.27 × 103/μL, respectively (P = 0.001). Mean CRP for OC and DNSOI were 104.4 mg/L and 10.0 mg/L, respectively (P < 0.001). The optimal CRP cut-off value of 20.2 mg/L demonstrated 90.9% sensitivity and 90.5% specificity (AUC = 0.946, P < 0.001) for differentiating between OC and DNSOI. CRP was more predictive of OC than WCC (P = 0.017). 7/26 (26.9%) OC patients with fever also had an elevated CRP, while 1/23 (4.3%) of DNSOI with fever had a normal CRP. Conclusions An elevated WCC is suggestive of OC. However, a normal WCC can neither exclude nor differentiate between OC and DNSOI. CRP may be a more accurate predictor of OC compared to WCC.

Identification of Hub Genes Associated with Nonspecific Orbital Inflammation by Weighted Gene Coexpression Network Analysis.

Background Nonspecific orbital inflammation is a common ophthalmopathy with a high prevalence among adult females. Yet, its molecular mechanisms behind are poorly understood. Regulation of gene expression probably plays an important role in this disease. Thus, we utilized gene coexpression networks to identify key modules and hub genes involved in nonspecific orbital inflammation. Methods Data of gene expression in nonspecific orbital inflammation samples (n = 61) and healthy samples (n = 28) were obtained from the public Gene Expression Omnibus database. Afterward, differentially expressed genes were performed. Then, weighted correlation network analysis was done to define the key modules. Next, functional enrichment analysis was conducted by Gene Ontology and Kyoto Encyclopedia of Genes and Genomes pathway in key modules. Finally, a protein-protein interaction network and Cytohubba plugin were used to screen hub genes. Results Differential expression of 716 genes was identified, among which 169 genes were upregulated and 547 genes were downregulated in the nonspecific orbital inflammation group. In weighted correlation network analysis, we clarified 2 key modules (MEturquoise and MEblue) that are likely to play key roles in nonspecific orbital inflammation. Functional enrichment analysis indicated that these genes are predominately involved in immune response and matrix homeostasis. In addition, among 2 key modules, there are 20 hub genes identified. Conclusion With this new approach, we identified several genes that could be critical to pathologies of nonspecific orbital inflammation. These findings may contribute to further therapeutic target development.

Misdiagnosis of fungal infections of the orbit

ObjectifÉvaluer l'exactitude du diagnostic initial des infections fongiques orbitaires et cerner les facteurs qui peuvent influer sur les résultats pour les patients. MéthodesUn examen rétrospectif des dossiers médicaux approuvé par le comité d’éthique d’établissement a été réalisé dans 2 importants hôpitaux universitaires afin de retracer les cas d'infections fongiques touchant l'orbite traités entre le 1er janvier 1998 et le 15 novembre 2019. Parmi les données recueillies, mentionnons les données démographiques des patients, leurs antécédents médicaux, les données recueillies lors de l'examen, le diagnostic, le traitement, l'imagerie et les résultats. RésultatsOn a recensé 50 cas d'infections fongiques orbitaires. De ce nombre, 33 (66,0 %) avaient d'abord été diagnostiquées à tort en tant qu'infections non fongiques. En outre, 16 patients (32,0 %) avaient reçu de multiples diagnostics initiaux, dont les plus fréquents, au moment de l'examen initial, étaient la cellulite bactérienne (n = 12 sur 50; 24,0 %) et la sinusite bactérienne (n = 12 sur 50; 24,0 %). Venaient ensuite les troubles vasculaires et inflammatoires de l'orbite (n = 9 sur 50; 18,0 %) : 5 patients (10,0 %) avaient reçu un diagnostic d'artérite temporale, 3 d'entre eux (6,0 %), un diagnostic d'inflammation orbitaire non spécifique et l'un d'eux (2,0 %), un diagnostic de névrite optique. Au sein de ce sous-groupe, 77,8 % (n = 7 sur 9) avaient d'abord reçu des stéroïdes par voie générale. Au nombre des autres diagnostics initiaux, citons les suivants : masse néoplasique, mucocèle, dacryocystite, thrombose du sinus caverneux, hémorragie, maladie transmise par les tiques, rhinite allergique et conjonctivite allergique. Il existe une corrélation significative entre les erreurs de diagnostic et une atteinte de l'espace masticateur visible à l'imagerie (p = 0,04). ConclusionLes infections fongiques orbitaires sont mal diagnostiquées dans 2 cas sur 3. Près de 15 % des patients chez lesquels on a ultérieurement diagnostiqué une infection fongique orbitaire avaient d'abord reçu des stéroïdes par voie générale. Les erreurs de diagnostic sont plus fréquentes en cas d'atteinte de l'espace masticateur.

Autoimmune markers in screening for orbital inflammatory disease

PurposeImmunogenic causes of inflammation may be difficult to differentiate in the work-up of orbital inflammatory disease. The study aims to investigate the utility of autoimmune markers in the screening for orbital inflammation. Markers studied included angiotensin-converting enzyme (ACE), antinuclear antibody (ANA), anti-neutrophilic cytoplasmic autoantibodies (ANCA), extractable nuclear antigen (ENA), anti-cyclic citrullinated peptide (Anti-CCP) and anti-double stranded DNA antibody (Anti-dsDNA antibody).MethodsA retrospective single-centre study of consecutive patients with non-infective orbital inflammation screened for autoimmune markers at presentation. Serology was interpreted alongside clinical course and other investigations (e.g. radiographic features and histopathology). Tabulated data and Pearson’s Chi-square allowed analysis of trends between serology, diagnosis and the decision to biopsy.Results79 patients, between 1999 and 2021, were included (50 females, mean age was 50.4 ± 17.4 years). 28 (34.6%) patients had specific orbital inflammation and 53 (65.4%) patients had non-specific orbital inflammation (NSOI). Of the 12 patients with positive serology and a specific diagnosis, only 5 (41.7%) patients had concordant serological results. There was no association between serology results and the patient undergoing biopsy (P = 0.651). Serology was unable to exclude nor differentiate NSOI from other specific conditions and ANA had limited discriminatory value between specific conditions and NSOI.ConclusionSerological testing alone may not provide a clear direction for further investigation of orbital inflammation and a biopsy may occur independently of the serological results. The value of autoimmune markers may lie in subsequent follow-up as patients may develop suggestive symptoms after an indeterminate positive result or initially seronegative disease.

Nonspecific orbital inflammation and thyroid eye disease, a rare comorbidity: report of two cases and review of literature

BackgroundTo present the very rare comorbidity of developing non-specific orbital inflammation (NSOI) in two patients with histories of definite thyroid eye disease (TED).Case presentationBoth patients complained of new-onset progressive proptosis although their thyroid disease was controlled and computed tomography scan revealed an intraorbital inflammatory mass. The pathological assessment indicated that both patients had developed fibrosing NSOI. Therefore, intravenous corticosteroids were administered. The mass regressed and the amount of proptosis was decreased in both patients.ConclusionsWe reviewed all related cases in the literature and extracted their clinical and radiological characteristics for this paper. Ophthalmologists should consider TED and NSOI in patients with a new-onset complaint of proptosis. Despite rare comorbidity of TED and NSOI, it should be considered especially in patients with refractory proptosis, and lead to its further evaluation and prompt management.

Periphlebitis of the Superior Ophthalmic Vein.

Isolated orbital periphlebitis in the context of nonspecific orbital inflammation is rare. This case series describes 3 patients with periphlebitis of the superior ophthalmic vein, 2 of which were confirmed with pathology. All 3 cases had a history of Graves hyperthyroidism. Superior ophthalmic vein periphlebitis is a rare form of orbital inflammation presenting with proptosis and motility restriction yet few inflammatory signs. It may be associated with autoimmune hyperthyroidism and represent a rare feature of thyroid eye disease, or perhaps could be an overlap syndrome between thyroid eye disease and nonspecific orbital inflammation. Treatment response is variable and may require a prolonged course of steroids or other immunosuppressive medications.

Enrichment of IGF-1R and PPARγ signalling pathways in orbital inflammatory diseases: steps toward understanding pathogenesis

BackgroundOrbital inflammatory disease (OID) encompasses a wide range of pathology including thyroid-associated orbitopathy (TAO), granulomatosis with polyangiitis (GPA), sarcoidosis and non-specific orbital inflammation (NSOI), accounting for up to 6% of...

Not a Tumor-Nonspecific Orbital Inflammation.

Objective This study was aimed to illustrate the features and complexities of nonspecific orbital inflammation via discussion of two representative cases. Design Present study is a retrospective case review. Setting The study was conducted at a tertiary care medical center. Participants Two patients with nonspecific orbital inflammation were participants of this retrospective study. Main Outcome Measures Outcome of the study was disease-free patients and off all medications. Results At follow-up, both patients are disease free and off all medications. Conclusion Surgery plays a diagnostic and therapeutic role. While the clinical subtype is important for differential diagnosis and symptomatic treatment, the histologic subtype is similarly important. For inflammatory dacryoadenitis, surgery can be therapeutic. For extensive granulomatosis with polyangiitis, debulking surgery may allow better penetration of medications, especially rituximab.

Posterior Scleritis Mimicking Orbital Cellulitis: A Report of the Three Cases

Objective: To report on three cases of posterior scleritis presenting as orbital cellulitis. Case Report: The authors retrospectively reviewed the medical records of three patients who were diagnosed as posterior scleritis but initially presented as orbital cellulitis. All patients were young males who presented with pain, unilateral proptosis, eyelid swelling with redness, and limitation of ocular movement. Initially, the diagnosis of orbital cellulitis was suspected and patients had been treated with systemic antibiotics but with no clinical improvement. Systemic work-up for infections and autoimmune diseases were negative. The orbital computed tomography (CT) scans showed severe posterior eyewall thickening with associated soft tissue edema. Ultrasonography showed a typical T-sign, compatible with posterior scleritis. Specific cause of scleritis or an association with systemic disease was not established. Systemic corticosteroids (1 mg per kg per day) were administered, and all patients improved within several days. Conclusion: Posterior scleritis is a rare and severe ocular inflammatory disorder that can mimic orbital cellulitis. Early detection and prompt treatment is crucial. Keywords: Posterior scleritis, Orbital cellulitis, Non-specific orbital inflammation, Orbital computed tomography scan, T-sign ultrasonography

Radiologic imaging shows variable accuracy in diagnosing orbital inflammatory disease and assessing its activity

Radiologic orbital imaging provides important information in the diagnosis and management of orbital inflammation. However, the diagnostic value of orbital imaging is not well elucidated. This study aimed to investigate the diagnostic accuracy of orbital imaging to diagnose orbital inflammatory diseases and its ability to detect active inflammation. We collected 75 scans of 52 patients (49 computed tomography (CT) scans; 26 magnetic resonance (MR) imaging scans). Clinical diagnoses included thyroid eye disease (TED) (41 scans, 31 patients), non-specific orbital inflammation (NSOI) (22 scans, 14 patients), sarcoidosis (4 scans, 3 patients), IgG4-related ophthalmic disease (IgG4-ROD) (5 scans, 3 patients), and granulomatosis with polyangiitis (GPA) (3 scans, 1 patient). Two experienced neuroradiologists interpreted the scans, offered a most likely diagnosis, and assessed the activity of inflammation, blinded to clinical findings. The accuracy rate of radiological diagnosis compared to each clinical diagnosis was evaluated. Sensitivity and specificity in detecting active inflammation were analyzed for TED and NSOI. The accuracy rate of radiologic diagnosis was 80.0% for IgG4-ROD, 77.3% for NSOI, and 73.2% for TED. Orbital imaging could not diagnose sarcoidosis. Orbital CT had a sensitivity of 50.0% and a specificity of 75.0% to predict active TED using clinical assessment as the gold standard. The sensitivity/specificity of orbital MR was 83.3/16.7% for the detection of active NSOI. In conclusion, orbital imaging is accurate for the diagnosis of IgG4, NSOI, and TED. Further studies with a large number of cases are needed to confirm this finding, especially with regard to uncommon diseases. Orbital CT showed moderate sensitivity and good specificity for identifying active TED.

Azathioprine in orbital apex syndrome

Orbital Apex Syndrome (OAS) is commonly caused by specific inflammations, infections, neoplasms, iatrogenic, trauma, vascular and non-specific orbital inflammation (NSOI). OAS secondary to NSOI responds rapidly to corticosteroids. There are very few case reports on azathioprine as additional treatment modality in OAS secondary to NSOI. We are presenting a 42 years old female who had recurrent episodes of painful ophthalmoplegia, treated with steroid therapy. Patient was steroid dependant. Therefore, she was started on azathioprine 2-3mg/kg body weight for 36 weeks. She had complete remission of the painful ophthalmoplegia, with no further recurrence on 6 months follow-up.

Choroidal folds associated with non-specific orbital inflammation

Choroidal folds associated with non-specific orbital inflammation