Idiopathic Orbital Inflammation Research Articles

Orbital Mass Identified With Point-of-Care Ultrasound in a Child Presenting With Eyelid Swelling.

Point-of-care ultrasound was used in a child presenting to the emergency department with progressively worsening left eyelid swelling. The early use of ultrasound was helpful in detecting a soft tissue mass that is exhibiting pressure effect on the eyeball. Point-of-care ultrasound helped expedite advanced imaging and consultation with the specialist. The patient eventually had a biopsy and was diagnosed with idiopathic orbital inflammation.

Update on Treatment of Idiopathic (and Non-Idiopathic) Orbital Inflammation

Update on Treatment of Idiopathic (and Non-Idiopathic) Orbital Inflammation

Epidemiology of Orbital Inflammatory Disease: An AAO IRIS Registry Study

ABSTRACT Purpose The current study queries the American Academy of Ophthalmology (AAO) Intelligent Research in Sight (IRIS) registry for data on the epidemiology, work-up, and management patterns of autoimmune orbital inflammation. Methods Analysis and description of patient data from the IRIS registry between 2013 and 2019 reviewing patients with autoimmune or idiopathic orbital inflammation with filters based on International Classification of Disease (ICD) and Current Procedural Terminology (CPT) codes. Patients with thyroid eye disease, orbital cellulitis, and orbital abscess were excluded. Main Outcome Measures Demographic descriptions included gender, age, geographic region, and treatment. Sub-analysis was performed by assessing rates of imaging, biopsy, lab work-up, and diagnostic categories. Results In a final cohort of 20,584 patients, the mean age of onset of orbital inflammation was 51.7 years; 67% female; and 63% Caucasian, 21% unknown, 12% Black, 2.6% Asian, and 1.5% other. Only 49 had imaging, 78 had laboratory work-up, and 1,411 had biopsy codes. Treatment results showed 166 patients receiving antibiotics, 224 patients receiving steroids, and 35 patients receiving both. Conclusions This study assessed the epidemiology, diagnostic patterns, and treatment patterns for orbital inflammation through the AAO IRIS registry. Practise patterns suggest a relatively low overall rate of imaging and laboratory studies compared to biopsies, although this certainly under-represents the actual number of imaging and laboratory studies and exemplifies the inherent imprecision of using a large database. However, the methodology of this study provides a framework of approaching the IRIS registry for oculoplastic research.

A Case of Idiopathic Orbital Inflammation With Elevated Anti-Mumps Immunoglobulin M Antibody.

A 21-year-old female presented with periocular swelling, diplopia, and painful ophthalmoplegia in the OS. Orbital magnetic resonance imaging revealed an enhanced soft tissue mass involving the left medial rectus muscle. Laboratory test results revealed leukocytosis, elevated reactive C-reactive protein, and positive serum levels of anti-mumps immunoglobulin M (IgM) antibody without systemic manifestations of mumps infection. The clinical course was refractory, and the patient showed a poor response to high-dose steroids. An incisional biopsy revealed stromal fibrosis with focal lymphoid aggregates, indicating sclerosing inflammation. Myopathy of the medial rectus progressed to superior, inferior, and lateral recti involvement of the left orbit. Immunosuppressive agents, including steroids, were administered for 22 months after disease onset. The mumps IgM antibody level was positive for over 5 months and became negative upon testing performed 1 year after the first visit.

CASE REPORT: IDIOPATHIC SCLEROSING ORBITAL INFLAMMATION

Idiopathic sclerosing orbital inflammation (ISOI) also known as sclerosing orbital pseudotumor is a rare, idiopathic, chronic, slowly progressive orbital inflammation, characterized by scarring of the tissue that represents a mass effect. The latter leads to bulging of the eye, restriction of ocular movements and visual impairment up to potential loss of vision. It is a subtype of non-specific inflammatory orbital pseudotumor, which both radiologically and clinically mimics a malignant process or other orbital inflammatory disease, and therefore is challenging to diagnose and manage. This case highlights the challenges in diagnosis and management of ISOI.

Composition and diversity analysis of the TCR CDR3 repertoire in patients with idiopathic orbital inflammation using high-throughput sequencing

BackgroundIdiopathic orbital inflammation (IOI) is a nonspecific orbital inflammatory disease with the third highest prevalence among orbital diseases, and its pathogenesis is associated with T-cell-mediated immune responses. This study aimed to investigate the differences in T-cell receptor (TCR) expression between IOI patients and healthy subjects by high-throughput sequencing and to characterize TCR expression in patients with IOI and with respect to glucocorticoid response.MethodsA total of 19 subjects were enrolled in this study and were divided into the idiopathic orbital inflammation group (IOI group, n = 13) and the healthy control group (HC group, n = 6), and within the IOI group were further divided into the glucocorticoid therapy sensitive group (IOI(EF) group, n = 6) and the glucocorticoid therapy ineffective group (IOI(IN) group, n = 7) based on the degree of effectiveness to glucocorticoid therapy. High-throughput TCR sequencing was performed on peripheral blood mononuclear cells of IOI patients and healthy control individuals using 5’ RACE technology combined with Unique Identifier (UID) digital tag correction technology. The TCR CDR3 region diversity, sharing patterns, and differential sequences between the IOI and HC groups, and between the IOI(EF) and IOI(IN) groups were analyzed.ResultsIt was found that the diversity of TCR CDR3 in the IOI group was significantly lower than that in the HC group, and the frequency of V gene use was significantly different between groups. The diversity of TCR CDR3 in patients in the IOI(EF) group was significantly lower than that in patients in the IOI(IN) group, and the frequency of V and J gene use was significantly different between the IOI(EF) group and the IOI(IN) group. Additionally, we found 133 nucleotide sequences shared in all IOI samples and screened two sequences with higher expression from them.ConclusionsOur results suggested that abnormal clonal expansion of specific T-cells exists in IOI patients and that TCR diversity may had an impact on the prognosis of glucocorticoid-treated IOI. This study may contribute to a better understanding of the immune status of IOI and provide new insights for T-cell -associated IOI pathogenesis, diagnosis and treatment prediction.

Intralesional or intraorbital rituximab injection for the management of biopsy-proven idiopathic orbital inflammation involving the lacrimal gland

ObjectiveTo evaluate the efficacy of intralesional rituximab injection for the management of idiopathic orbital inflammation (IOI) involving the lacrimal gland, which is the most common subtype. MethodEighteen consecutive patients with biopsy-proven IOI involving the lacrimal gland were included. Rituximab (50 mg/5 mL) was injected intralesionally at monthly intervals. ResultsClinically, all patients presented with upper eyelid swelling and ptosis. Most patients (56%) had periocular pain and a palpable superotemporal mass. Biopsies showed chronic inflammation without fibrosis in 14 patients (78%) and chronic inflammation and fibrosis in 4 patients (22%). Intralesional rituximab was injected once in 1 patient (6%) because of complete response after the first injection, twice in 11 patients (61%), and 3 times in 6 patients (33%) because of partial response after 2 injections. After a mean follow-up of 33 months (median, 33 months; range, 11–59 months), 16 patients (89%) showed a clinical response, including 14 patients (78%) a complete response (i.e., disappearance of all lesions) and 2 patients (11%) with a partial response (i.e., ≤30% decrease in lesion diameter). Two patients (11%) did not respond after 3 injections and were placed on systemic corticosteroid and methotrexate therapies. Two patients (11%) with a complete response developed subsequent recurrence 12 and 49 months after their last injections. Both were treated with 2 additional rituximab injections, 1 month apart, and showed complete response when examined 27 and 11 months after treatment, respectively. ConclusionIntralesional rituximab injection may be an effective treatment for IOI involving the lacrimal gland, achieving a 78% complete response rate in this series. Local treatment with rituximab has the potential to avoid the ocular and systemic side effects of corticosteroid and systemic immunosuppressive treatment.

Limited granulomatosis with polyangiitis in children with idiopathic orbital inflammation: a case series and literature review.

Idiopathic orbital inflammation (IOI) is a diagnosis of exclusion, but the exclusion of other inflammatory diseases of the orbit is broad and relies on clinician experience, response to corticosteroid, or biopsy. This study aimed to investigate the presence of granulomatosis with polyangiitis (GPA) in patients initially diagnosed as IOI and describe its clinicopathological features, ANCA status, treatment, and outcome. We performed a retrospective case series study of children diagnosed with limited GPA (L-GPA) in patients with IOI. A systematic review of the literature was performed in children with GPA and orbital mass. Eleven of 13 (85%) patients with IOI had L-GPA. Two additional patients with orbital mass and L-GPA were included in this analysis. The median age was 10years, and 75% were female. Twelve cases were ANCA positive and 77% were MPO-pANCA positive. Most patients had a poor response to treatment and had a high relapse rate. Based on literature review, 28 cases were found. Most (78.6%) were female with a median age of 9years. Three patients were misdiagnosed as IOI. Patients with L-GPA more frequently had MPO-pANCA positivity (35%) than children with systemic GPA (18%) and were less often PR3-cANCA positive than patients with systemic GPA (18% vs. 46%). L-GPA accounts for a high prevalence of children diagnosed as IOI. The high prevalence of MPO-pANCA observed in our study may be related to L-GPA rather than with the orbital mass. Long-term follow-up, orbital biopsy, and serial ANCA testing are necessary to exclude GPA in patients with IOI.

Not all orbital inflammations in the COVID-19 era are due to mucormycosis – A case of orbital pseudotumour leading to vision loss

A 57-year-old male patient with type 2 diabetes mellitus who had survived severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) disease (COVID-19) presented with loss of vision in the right eye (RE) of 20-day duration. The patient had been diagnosed elsewhere with orbital mucormycosis and had been treated for it. The patient developed vision loss in RE 1 week after the initiation of treatment. At the time of presentation in our institute, vision in RE had reduced to no perception of light. Examination showed a relative afferent pupillary defect and pale optic disc in RE. Gadolinium-enhanced contrast magnetic resonance imaging of the brain, orbit and paranasal sinuses suggested perineuritic type of idiopathic orbital inflammation. In the present COVID-19 scenario, diagnosis other than mucormycosis should be kept in mind when dealing with cases of orbital inflammation.

Acute Orbital Apex Syndrome Caused by Idiopathic Sclerosing Orbital Inflammation.

Idiopathic sclerosing orbital inflammation (ISOI) is a distinct entity among other orbital diseases. It is characterized by marked fibrosis and inflammatory cell infiltration that can damage orbital structures. Clinical manifestations were variable, including ocular and periocular redness, proptosis, and pain. Ocular motor restrictions and optic nerve dysfunction might occur in severe cases. We herein report a patient of ISOI who presented with total ophthalmoplegia and acute vision loss. His symptoms were relieved mainly as his vision improved to 20/25 after receiving corticosteroid and immunosuppressant therapies. Therefore, ISOI should be one of the deferential diagnoses when we encounter cases with acute orbital apex syndrome. With prompt evaluation and in-time treatment, a favorable outcome is possible.

MRI-Based Radiomics Nomogram for Preoperative Differentiation Between Ocular Adnexal Lymphoma and Idiopathic Orbital Inflammation.

Ocular adnexal lymphoma (OAL) and idiopathic orbital inflammation (IOI) are malignant and benign lesions for which radiotherapy and corticosteroids are indicated, but similar clinical manifestations make their differentiation difficult. To develop and validate an MRI-based radiomics nomogram for individual diagnosis of OAL vs. IOI. Retrospective. A total of 103 patients (46.6% female) with mean age of 56.4 ± 16.3 years having OAL (n=58) or IOI (n=45) were divided into an independent training (n=82) and a testing dataset (n=21). A 3-T, precontrast T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), and postcontrast T1WI (T1 + C). Radiomics features were extracted and selected from segmented tumors and peritumoral regions in MRI before-and-after filtering. These features, alone or combined with clinical characteristics, were used to construct a radiomics or joint signature to differentiate OAL from IOI, respectively. A joint nomogram was built to show the impact of the radiomics signature and clinical characteristics on individual risk of developing OAL. Area under the curve (AUC) and accuracy (ACC) were used for performance evaluation. Mann-Whitney U and Chi-square tests were used to analyze continuous and categorical variables. Decision curve analysis, kappa statistics, DeLong and Hosmer-Lemeshow tests were also conducted. P < 0.05 was considered statistically significant. The joint signature achieved an AUC of 0.833 (95% confidence interval [CI]: 0.806-0.870), slightly better than the radiomics signature with an AUC of 0.806 (95% CI: 0.767-0.838) (P=0.778). The joint and radiomics signatures were comparable to experienced radiologists referencing to clinical characteristics (ACC=0.810 vs. 0.796-0.806, P > 0.05) or not (AUC=0.806 vs. 0.753-0.791, P > 0.05), respectively. The joint nomogram gained more net benefits than the radiomics nomogram, despite both showing good calibration and discriminatory efficiency (P > 0.05). The developed radiomics-based analysis might help to improve the diagnostic performance and reveal the association between radiomics features and individual risk of developing OAL. 3 TECHNICAL EFFICACY: 3.

Proteotranscriptomics of ocular adnexal B-cell lymphoma reveals an oncogenic role of alternative splicing and identifies a diagnostic marker

BackgroundOcular adnexal B-cell lymphoma (OABL) is a rare subtype of non-Hodgkin lymphoma. The molecular characteristics of OABL remain poorly understood. We performed an integrated study to investigate the proteotranscriptome landscape and identify novel molecular characteristics and biomarkers of OABL.MethodsIntegrated quantitative proteome and transcriptome were performed on 40 OABL 12 idiopathic orbital inflammation, 6 reactive lymphoid hyperplasia, and 13 aesthetic orbital plastic surgery specimens. Complete clinicopathologic and prognostic data of the patients were recorded.ResultsWe identified high global protein-mRNA concordance as a novel characteristic of OABL. High concordance was related to OABL recurrence. By integrated expression profile, motif enrichment and trend analysis, we found that alternative splicing is inflammation-independently dysregulated in OABL. After portraying the aberrant alternative splicing event landscape, we demonstrated the oncogenic role of ADAR, a core splicing regulator that regulates the splicing of Rho GTPase and cell cycle members. We found that ADAR regulates cell proliferation and Rho GTPase inhibitor sensitivity of lymphoma. We identified DNAJC9 as a potential biomarker for OABL in proteomic analyses. Immunohistochemistry and immunofluorescent staining showed the nuclear staining of DNAJC9 was significantly higher in extranodal marginal zone lymphomas compared with inflammation specimens.ConclusionsThese results provide an integrated gene expression profiling and demonstrate that high global protein-mRNA concordance is a prognosis-related molecular characteristic of OABL. We portray the alternative splicing events landscape of OABL, and reveal the oncogenic role of ADAR. We identified strong nuclear staining of DNAJC9 as a promising pathology diagnostic biomarker for extranodal marginal zone lymphomas.

Canine idiopathic orbital inflammation: A case series of four affected dogs (five orbits)

In humans, idiopathic orbital inflammation (IOI) is a diagnosis attributed to benign, inflammatory orbital conditions without identifiable local or systemic cause. We describe the clinical signs, imaging and histopathological findings, management and outcome of four dogs diagnosed with IOI. Multicentric retrospective study. A total of four dogs (five orbits) of three different breeds (three cases were English Springer Spaniels [ESS] or ESS-cross) and ages ranging from 3 to 12 years were included. Initial presenting signs were unilateral and included exophthalmos, enophthalmos, globe deviation, thickening and protrusion of the third eyelid and conjunctival hyperemia. Computed tomography and magnetic resonance imaging identified heterogeneous space-occupying, contrast-enhancing orbital lesions in all cases. Sparing of the retrobulbar space was detected in four of five orbits. Histopathology revealed mixed inflammatory infiltrates of lymphocytes, plasma cells, and histiocytes. Immunohistochemistry was performed in two cases highlighting the presence of histiocytes and lymphocytes, predominantly T cells. Resolution of clinical signs was achieved in two cases managed with oral immunosuppressant medication (corticosteroids alone or combined with cyclosporine or azathioprine), one went into spontaneous remission, one resolved with topical corticosteroids, and one underwent exenteration. Recurrence occurred in two cases within 15months of initial diagnosis and required further immunosuppressant medication. One case developed signs in the contralateral orbit within 8months of presentation. IOI is an uncommon condition in dogs. Its diagnosis relies on the combination of advanced imaging and histology. As in humans, it appears that spontaneous remission and recurrence may occur requiring long-term immunosuppressant medication.

A deep learning model combining multimodal radiomics, clinical and imaging features for differentiating ocular adnexal lymphoma from idiopathic orbital inflammation.

To evaluate the value of deep learning (DL) combining multimodal radiomics and clinical and imaging features for differentiating ocular adnexal lymphoma (OAL) from idiopathic orbital inflammation (IOI). Eighty-nine patients with histopathologically confirmed OAL (n = 39) and IOI (n = 50) were divided into training and validation groups. Convolutional neural networks and multimodal fusion layers were used to extract multimodal radiomics features from the T1-weighted image (T1WI), T2-weighted image, and contrast-enhanced T1WI. These multimodal radiomics features were then combined with clinical and imaging features and used together to differentiate between OAL and IOI. The area under the curve (AUC) was used to evaluate DL models with different features under five-fold cross-validation. The Student t-test, chi-squared, or Fisher exact test was used for comparison of different groups. In the validation group, the diagnostic AUC of the DL model using combined features was 0.953 (95% CI, 0.895-1.000), higher than that of the DL model using multimodal radiomics features (0.843, 95% CI, 0.786-0.898, p < 0.01) or clinical and imaging features only (0.882, 95% CI, 0.782-0.982, p = 0.13). The DL model built on multimodal radiomics features outperformed those built on most bimodalities and unimodalities (p < 0.05). In addition, the DL-based analysis with the orbital cone area (covering both the orbital mass and surrounding tissues) was superior to that with the region of interest (ROI) covering only the mass area, although the difference was not significant (p = 0.33). DL-based analysis that combines multimodal radiomics features with clinical and imaging features may help to differentiate between OAL and IOI. • It is difficult to differentiate OAL from IOI due to the overlap in clinical and imaging manifestations. • Radiomics has shown potential for noninvasive diagnosis of different orbital lymphoproliferative disorders. • DL-based analysis combining radiomics and imaging and clinical features may help the differentiation between OAL and IOI.

Clinical heterogeneity between two subgroups of patients with idiopathic orbital inflammation

ObjectiveIdiopathic orbital inflammation (IOI) is a group of orbital inflammatory diseases of unknown etiopathogenesis. We investigated whether patients with IOI have clinical heterogeneity based on the presence (typical group) or...

Clinical Characteristics of Idiopathic Orbital Inflammation Syndrome in Relation to Intraocular Pressure.

PurposeTo investigate the clinical characteristics of idiopathic orbital inflammation and changes in intraocular pressure (IOP) before and after its treatment.Patients and MethodsWe retrospectively studied 20 eyes from the medical records of 19 patients who were diagnosed with idiopathic orbital inflammation between April 1, 2004, and April 30, 2019, at Toyama University Hospital. The inflammation site (type of disease), treatment provided, IOP before and after treatment, and the symptoms (proptosis, decreased ocular movements or diplopia, periorbital edema, and ocular pain) were analyzed.ResultsThe types of idiopathic orbital inflammation were dacryoadenitis in 14, myositis in 7, diffuse-type in 3, and posterior periscleritis in 1 case. The mean IOP after treatment was 15.4±3.9 mm Hg, which was significantly lower than the mean pretreatment IOP of 19.0±5.3 mm Hg (p = 0.009). Before treatment, all cases with the diffuse-type had high IOPs of 21 mm Hg or more. Ocular pain and eye movement disorders were present in 86% and 100% of subjects in the group with an IOP of 21 mm Hg or higher, but 38% and 31% in the group with an IOP of 20 mm Hg or lower, respectively.ConclusionDiffuse-type of idiopathic orbital inflammation is prone to develop high IOP. Patients with idiopathic orbital inflammation and high IOP exhibit many symptoms such as decreased ocular movements, diplopia, and ocular pain.

Idiopathic Orbital Inflammation in the Postpartum Period Associated With Preeclampsia.

Idiopathic Orbital Inflammation in the Postpartum Period Associated With Preeclampsia.

[Bilateral idiopathic orbital inflammation with chronic sinusitis-an IgG4-related disease?]

[Bilateral idiopathic orbital inflammation with chronic sinusitis-an IgG4-related disease?]

Imaging Findings of Pediatric Orbital Masses and Tumor Mimics.

Pediatric orbital masses are not common but encompass a wide spectrum of benign and malignant entities that range from developmental anomalies to primary and secondary orbital malignancies and metastatic disease. Certain orbital tumors are unique to pediatric patients, such as retinoblastoma and neuroblastoma. Clinical symptoms and signs are often insufficient to differentiate between orbital lesions, and imaging is essential for narrowing the diagnostic considerations and determining the most appropriate management strategy. MRI is the primary imaging modality for evaluating orbital masses in children, with US and CT playing complementary roles. The authors review a spectrum of masses and tumor mimics that affect the pediatric globe and orbit. The shared and differentiating characteristics of pediatric orbital lesions are reviewed. Emphasis is placed on utilizing an orbital compartment-based approach to narrow the differential diagnosis. By using this organizational scheme, the authors describe intraocular processes (retinoblastoma, persistent fetal vasculature, and Coats disease), intraconal lesions (lymphatic malformation, schwannoma, optic nerve sheath meningioma, and optic pathway glioma), extraconal lesions (infantile hemangioma, rhabdomyosarcoma, idiopathic orbital inflammation, lymphoma, venous varix, plexiform neurofibroma, and pleomorphic adenoma of the lacrimal gland), and lesions involving the bony orbit (dermoid cyst, metastatic neuroblastoma, and Langerhans cell histiocytosis). The authors describe the basic management of each entity. Orbital infections and traumatic lesions are beyond the scope of this article. ©RSNA, 2022.

P30 A case of recurrent orbital myositis

Case report - IntroductionAn interesting case of an 11-year recurrent isolated lateral rectus myositis. Initially responsive to short courses of oral steroids; however, this has more recently become refractory and now the patient is unable to wean off steroids. The use of biologics in these patients is uncommon and there are very few case reports of this and the outcomes. We present a case of refractory orbital myositis treated with rituximab.Case report - Case descriptionA 38-year-old female presented in 2009 with ocular pain, swelling and diplopia. After review by the ophthalmology team an MRI orbits was requested which showed swelling and enhancement of the right lateral rectus muscle, consistent with orbital myositis. She had no other symptoms of myositis elsewhere, and no other symptoms suggestive of a connective tissue disease. Her ANA, ENA and extended myositis panel were negative and therefore aetiology was uncertain. She had no past medical history, but was later diagnosed with hypothyroidism with positive TPO antibodies. Throughout the 11 years since diagnosis her thyroid disease has been well controlled, and she has had normal free T3/T4 and TSH. It was felt that thyroid eye disease was unlikely to present with a single muscle. She was initially treated with a course of 40mg oral prednisolone reducing over 6 weeks and symptoms resolved. In 2013 she had her first relapse, which was treated with prednisolone reducing over the course of 12months, and again symptoms resolved. Her third relapse in 2019 was treated with steroids, but patient was unable to wean off this, requiring 60mg of prednisolone, and at this point was referred to rheumatology for steroid sparing agents. Between 2013 and 2019 there was MRI progression with volumetric enlargement of the right lateral rectus with intra-orbital space reduction.Under the care of rheumatology, she had repeat connective tissue disorders and myositis screen. She was started on azathioprine as a steroid sparing agent. This had little effect and she was still debilitated by orbital pain and diplopia, and unable to reduce her prednisolone below 30mg daily. She trialled intra-orbital steroid injections with little benefit. She has now been started on rituximab infusions to allow us to wean steroids. Thus far she has had two doses and we await the outcome from this treatment. Case report - DiscussionOrbital myositis can be a debilitating condition causing diplopia and pain, as in the case of this 38-year-old female who has been off work for the past year due to the condition. It can affect single muscles or multiple muscles and may be unilateral or bilateral. The major differential diagnosis is thyroid eye disease which would not usually cause an isolated myopathy, is usually painless and slowly progressive and as such was felt unlikely to be the underlying pathology in this case. From a literature review into idiopathic orbital inflammation, including myositis, 75% of patients are found to have a good response to corticosteroids. Second-line treatments include radiotherapy, methotrexate/azathioprine and other biologic agents. A case report in 2014 of 10 patients with orbital myositis refractory to steroids and at least one other immunosuppressant, demonstrated that rituximab was safe and effective, with 7/10 patients noting improvement of their symptoms. Out of those seven patients, four had been on steroids at induction of rituximab and all of the patients were able to reduce their steroid dose. The patients in this trial received two initial doses of rituximab and were permitted to have a further dose at 24 weeks if there were recurrence of symptoms. Four out of the seven patients required a further infusion after 24 weeks. In a review of the literature, we have noted a further two case reports of the use of rituximab in orbital myositis from 2008 and 2012 with good response. Both patients were unable to wean from corticosteroids and had tried other DMARDs such as methotrexate. Other case reports of biologics therapy include a case report of two patients in which adalimumab was used and allowed steroid reduction with good results for at least 9 months.Case report - Key learning pointsOrbital myositis should be assessed for other features of polymyositis, as well as investigated for thyroid eye diseaseMost patients will have a good response to oral corticosteroidsAround a quarter of cases can have refractory disease. In these patients there remains a management dilemma. Options include radiotherapy, disease modifying agents such as methotrexate and azathioprine, and biologic therapy such as adalimumab or rituximabThere is limited evidence in the literature as to the best treatment option and ideal length of treatment for refractory orbital myositis cases