Myasthenia gravis-inflammatory myopathy (MG-IM) overlap syndrome can occur as an immune-related adverse event (irAE) after immune checkpoint inhibitor (ICI) therapy. Before the advent of ICIs, a sporadic form of MG-IM (s-MG-IM), often thymoma-associated, was already described. This study compared clinical and serologic features, treatment strategies, and outcomes of s-MG-IM and ICI-related MG-IM (ir-MG-IM). We conducted a multicenter, retrospective cohort study across 8 Italian centers, including consecutive patients fulfilling established criteria for both MG and IM, with or without previous exposure to ICIs. Clinical features, antibody profiles, oncologic history, treatments, and outcomes were collected and compared. Available serum samples were tested for binding capacity and pathogenic properties (antigenic modulation and complement-activating capacity) against clustered adult (A) and fetal (F) AChR isoforms using a live cell-based assay (L-CBA). Thirty-eight patients were identified (19 with s-MG-IM and 19 with ir-MG-IM). The ir-MG-IM cohort was older (median 77 vs 59 years, p < 0.001), predominantly male (84.2% vs 47.4%), and exclusively presented with concurrent MG/IM onset, whereas s-MG-IM often manifested sequentially. Thymoma was detected in 73.7% of patients with s-MG-IM but in none of the ir-MG-IM group. CK levels were higher in the ir-MG-IM cohort (median 3,145 vs 1,000 U/L, p = 0.001). Myocarditis, orbital myositis, and severe bulbar/respiratory involvement were more frequent in the ir-MG-IM cohort (p < 0.05). Anti-AChR antibodies were found in all patients with s-MG-IM and in 68% of patients with ir-MG-IM. Exploratory in vitro assays showed antigenic modulation and complement activation in s-MG-IM serum samples (8/9), whereas no activity was detected in the ir-MG-IM samples tested (0/4). Anti-titin antibodies were identified in 85.7% of patients with s-MG-IM (all thymoma-associated) and 50% of patients with ir-MG-IM. Acute mortality occurred in 36.8% of patients with ir-MG-IM and in no patients with s-MG-IM (p < 0.01). Relapses occurred in 63.2% of patients with s-MG-IM but were absent in patients with ir-MG-IM (p < 0.001). Although they share some similarities, s-MG-IM represents a chronic, predominantly thymoma-associated overlap syndrome with classical MG and IM features, whereas ir-MG-IM is typically an aggressive, likely monophasic condition characterized by severe myositis, with frequent ocular and cardiac involvement, and lacking classical MG features. Study limitations include the retrospective design, small sample size, and limited serum availability, warranting confirmation in larger prospective cohorts.

Extra-intestinal manifestations of inflammatory bowel disease (IBD) occur in 25-40% of patients; however, orbital involvement is rare and underreported. Only case reports and series have been documented in the literature with no consensus on diagnosis or management. This systematic review aims to compile and analyse the published literature on orbital manifestations of IBD, focusing on clinical presentation, imaging, management and outcomes. A systematic search of PubMed, Embase, and Web of Science was performed from database inception to April 2025. Data were collected and risk of bias was assessed using the JBI Critical Appraisal Checklist for Case Reports/Case Series. 41 studies reporting 48 patients were included. Mean age at orbital presentation was 31 years (range 2-63 years) with a female predominance (3:1). Orbital inflammation preceded IBD diagnosis in 19 patients. Crohn's disease was most frequently associated (n = 40). The most common clinical features were periorbital pain, swelling and ophthalmoplegia, with orbital myositis (n = 32) and dacryoadenitis (n = 15) being the predominant manifestations. 30 of 48 cases (63%) presented with bilateral disease, in contrast to the 8-20% bilateral rate typically reported for non-specific orbital inflammation (NSOI). Imaging most often demonstrated extraocular muscle enlargement. The majority of patients received systemic corticosteroids, often in combination with immunomodulators or biologics with most achieving remission. Recurrence occurred in 22 patients, and two experienced permanent vision loss. Orbital IBD is a rare but clinically significant entity, and may precede intestinal symptoms, sometimes representing the first sign of underlying IBD. Clinicians should maintain vigilance for orbital involvement in IBD, and standardised prospective reporting is needed to better characterise these manifestations and optimise patient outcomes.

A 70-year-old woman diagnosed with nodal T follicular helper cell lymphoma, angioimmunoblastic type (nTFHL-AI), was treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). Approximately 2 weeks after the fifth cycle of CHOP, the patient developed difficulty in keeping her right eye open. Simultaneously, cervical lymphadenopathy was observed. Eye examination revealed solid subconjunctival tissue, chemosis, conjunctival hyperemia, eyelid redness/swelling, and diplopia (Figure A,B). Magnetic resonance imaging revealed contrast enhancement in the soft tissue surrounding the right eyeball (Figure C). Positron emission tomography revealed hypermetabolic masses on the right eyelid and eyeball (Figure D). Conjunctival biopsy was performed, and tissue samples were obtained from the inferonasal and inferotemporal conjunctiva. Hematoxylin and eosin-stained sections demonstrated the infiltration of small- to medium-sized lymphoid cells into the lamina propria (Figure E). Immunohistochemical analysis revealed that the lymphoid cells were positive for CD3, CD4, CD5, BCL-6, PD-1, ICOS, and CXCL-13 (Figure F–H) and negative for CD8, CD20, CD30, and EBER, which were matched as the IHC profile of nTFHL-AI. Based on these findings, the conjunctival lesion was diagnosed as an nTFHL-AI relapse. Cervical lymph node biopsy also confirmed nTFHL-AI relapse and identified clonal T-cell receptor-β gene rearrangement. The patient received salvage chemotherapy with ifosfamide, carboplatin, and etoposide, which proved ineffective. In contrast, the patient responded to involved-site radiation therapy of the right eye combined with tucidinostat, an oral histone deacetylase inhibitor. Ocular adnexal lymphoma accounts for 1%–2% of all non-Hodgkin lymphomas, most of which are of the B-cell lineage [1]. Conjunctival T-cell lymphoma is rare, with conjunctival nTFHL-AI being extremely uncommon [2, 3]. Conjunctival lymphomas exhibit distinct ocular features by subtype and are particularly useful for diagnosing common B-cell lymphomas [2]. Conversely, ocular manifestations are often insufficiently described in T-cell lymphomas, which are rare subtypes of conjunctival lymphomas [3-7]. In a previously reported case of nTFHL-AI, the ocular findings comprised two pink, discrete, non-tender bulbar conjunctival masses [3]. In our case, in addition to a solid conjunctival mass, we observed chemosis, conjunctival hyperemia, eyelid redness and swelling, and diplopia. Our case presents a rare manifestation of ocular involvement of nTFHL-AI and provides insights into the differential diagnosis of a red eye, which includes both benign and serious conditions requiring ophthalmologic evaluation, including infections, rheumatologic disorders, thyroid diseases, sarcoidosis, orbital myositis, and, rarely, malignancies. The authors have nothing to report. The authors have nothing to report. The authors have nothing to report. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. The authors declare no conflicts of interest. Data from this study are not deposited in a publicly accessible database.

Vacuoles, E1-ligase, X-linked Auto-inflammatory, Somatic (VEXAS) syndrome is a recently described multisystem inflammatory disorder. Ocular features are described, though not well known; we aimed to describe them. This is a case series of VEXAS patients submitted by ophthalmologists from relevant specialty organisations, including the Australian and New Zealand Society of Ophthalmic Plastic Surgeons, British Oculoplastic Surgical Society and the Orbital Society. Patient consent was received. 14 males with VEXAS syndrome and eye features were included, median age 73 years (range 49-78). The associated UBA1 mutation was most commonly p.Met41Val (n=8, 57%), and most patients were Caucasian (n=11, 79%). All patients reported eyelid swelling (n=14, 100%); the next most frequent symptom was eye pain (n=10, 71%). Eye symptoms showed variable duration at presentation, most commonly 2-7 days (n=6, 43%). Eye involvement was typically bilateral (metachronous n=5, 36%, synchronous n=2, 14%). Overall, visual acuity was normal and did not change. Severe vision loss occurred unilaterally in two patients (14%), due to orbital compartment syndrome and periorbital necrotising fasciitis. Ophthalmologist-reported clinical features included periorbital oedema, present in all cases (n=14, 100%), followed by dacryoadenitis (n=8, 57%) and orbital myositis (n=7, 50%). There were no cases of posterior segment inflammation. Death due to complications of VEXAS occurred in one patient (7%). Orbital inflammation was a feature in all cases of VEXAS with eye involvement, and severe loss of vision occurred unilaterally in 14%. Ocular complaints in VEXAS patients should prompt urgent ophthalmic assessment.

BackgroundOrbital myositis (OM) is an uncommon ocular manifestation of systemic lupus erythematosus (SLE) and is typically characterized by acute painful proptosis and diplopia. Only isolated painful cases have been documented, and these usually occur without systemic activity.CaseA 44-year-old woman with a six-year history of SLE developed painless bilateral proptosis and mild abduction restriction during a severe multisystem flare involving the kidneys, lungs, and gallbladder. Laboratory studies revealed high anti-dsDNA titres, hypocomplementemia, and elevated inflammatory markers. Orbital computed tomography demonstrated isolated enlargement of both lateral rectus muscles without evidence of infection, sinus disease, or thyroid orbitopathy. She received intravenous methylprednisolone 1000mg daily for 3 days, followed by an oral prednisone taper (1mg/kg/day) and intravenous cyclophosphamide 1000mg every 4 weeks for six pulses. Both ocular and systemic manifestations resolved within 24 hours of initiating pulse therapy. During admission, she developed acalculous cholecystitis, attributed to active lupus vasculitis after multidisciplinary assessment.ConclusionThis represents the first reported case of painless orbital myositis associated with systemic lupus erythematosus during a multi-systemic flare. Rapid response to corticosteroid and cyclophosphamide therapy underscores the inflammatory, reversible nature of this manifestation. Recognition of OM as a potential lupus complication is critical for prompt diagnosis, exclusion of infectious causes, and early initiation of aggressive immunosuppression to preserve vision and prevent systemic morbidity.

Missed opportunities for diagnostic excellence are common and can lead to delayed diagnoses and initiation of treatment. Medical teams incorporate many elements into the diagnostic process, including patient factors, medical knowledge, data gathering, care environment, subspecialized personnel, and hospital processes. A case-based discussion describes how search satisfying-the tendency to stop searching once something has been found-and authority gradients can lead to delays in diagnosis. A 2-year-old girl repeatedly presents to the emergency department with vomiting and periorbital swelling progressing to esotropia, initially found to have normal short-sequence MR brain imaging. After nonspecific labs and reassuring initial imaging, ophthalmologic consultation obtained during the child's third emergency department visit proposed plausible diagnoses of benign post-viral esotropia or decompensated esotropia. When her exam worsened at ophthalmology clinic follow up, she was referred back to the emergency department for complete MR brain and orbit imaging leading to a diagnosis of bilateral orbital myositis. Examining the diagnostic process through integrated commentary, this case emphasizes the importance of recognizing limitations of short-sequencing advanced imaging and continuing the diagnostic pursuit in collaboration with consultants. A fishbone diagram visually demonstrates the factors that lead to a missed opportunity for diagnostic excellence. The case concludes with clinical teaching points in addition to a pitfall, myth, and pearl specific to search satisfying and authority gradients.

ABSTRACT Purpose To compare the clinical presentation, imaging features and outcomes of patients with orbital myositis (OM) and thyroid associated orbitopathy (TAO). Methods A retrospective and comparative study of 60 patients diagnosed with OM in a tertiary Eye Clinic between 2012 and 2023. Cases were matched with 60 TAO controls by sex and age. Results Clinical presentation of OM differs from TAO with a unilateral involvement and an acute onset of symptoms in 93% and 65% of cases, respectively. OM usually affect a single extraocular muscle (63%), typically the medial rectus, whereas TAO affect multiple and several EOM, with higher incidence of increased orbital fat volume. Tendon sparing on MRI was not discriminant between both conditions. Patients with OM had a better remission rate at 12 months (48% vs 14%, p < 0.001). Optic neuritis occurred in only one patient with OM vs. 11 patients with TAO (p < 0.001) and 12 patients had a relapse of OM with a contralateral involvement in 3/12 patients (25%). Conclusions This comparative study highlights key clinical and imaging features that distinguish OM from TAO. OM is associated with a better clinical outcome and prognosis with a reduced risk of optic neuritis and relapse compared with TAO patients.

Inflammatory bowel disease (IBD) is an autoimmune inflammatory condition of the gastrointestinal tract and encompasses 2 major subtypes: Crohn's disease and ulcerative colitis. Orbital myositis is an uncommon ophthalmic extraintestinal manifestation of IBD and may be associated with an internal necrotic collection. The presence of a necrotic collection in orbital myositis narrows the diagnostic differential to several infectious and noninfectious etiologies. The distinction between an infectious and noninfectious etiology is of significant therapeutic consideration, as management differs greatly. We present a case of a 17-year-old female presenting with a left lateral rectus orbital myositis associated with an internal necrotizing collection of the left lateral rectus. Histopathology demonstrated severe nonspecific chronic orbital myositis with features suggestive of focal granulomatous inflammation. She had an elevated fecal calprotectin and proceed to a colonoscopy, ultimately confirming a diagnosis of Crohn's disease. She was subsequently commenced on oral azathioprine and an oral prednisolone tapering course with ongoing follow-up. Her orbital disease remains quiescent at 6 months following her diagnosis.

Scleroderma-polymyositis overlap syndrome (SSc-PM) is an uncommon autoimmune condition characterised by clinical and serological features of both systemic sclerosis and inflammatory myopathy. Ocular manifestations of this syndrome are rare and insufficiently described in the literature. We report a case of SSc-PM presenting with orbital myositis as the primary manifestation, accompanied by proximal myopathy. A middle-aged woman presented with a 3-month history of progressive finger skin tightening, painless diplopia and restricted eye movements. Ophthalmological evaluation and imaging confirmed bilateral lateral rectus myositis. Laboratory investigations revealed elevated creatine kinase, positive antinuclear antibodies and anti-Ku antibody positivity. Following the rheumatology consultation, she was treated with intravenous methylprednisolone, transitioned to tapering oral prednisolone and initiated on mycophenolate mofetil. Her diplopia resolved completely and muscle enzyme levels normalised. Over 6 months of follow-up, there was no progression of cutaneous or pulmonary disease. This case highlights orbital myositis as a rare but treatable manifestation of SSc-PM, underscoring the importance of early recognition and prompt immunosuppression in overlap syndromes.

Idiopathic orbital myositis is a rare inflammatory condition that predominantly affects multiple extraocular muscles. Isolated involvement of a single muscle is very uncommon. Isolated levator palpebrae myositis is a diagnostic challenge that should be considered in patients with periocular pain and complete eyelid ptosis without ophthalmoparesis or pupillary dysfunction. We propose a comprehensive set of diagnostic tests to identify the underlying etiology and a recommended treatment regimen. Additionally, we present a case from our clinical practice that illustrates this rare presentation, of which few cases have been described in the literature.

To identify and analyze ocular features seen in Vacuoles, E1-ligase, X-linked Auto-inflammatory, Somatic (VEXAS) syndrome. A systematic literature review was performed following PRISMA guidelines (PROSPERO registration number: ID 566167). Article inclusion criteria comprised genetic confirmation VEXAS syndrome that included eye involvement. Exclusion criteria included lack of genetic testing, or ocular feature reporting. A systematic search of the PubMed/MEDLINE, Embase, and CENTRAL databases was performed from January 2020 to September 2024. Data were collected and risk of bias assessed in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. For the meta-analysis, specific UBA1 mutation and systemic feature data were also included. An association between severity of ocular features, presence of specific ophthalmic or systemic features, with age or causative mutation was investigated using Kruskal-Wallis rank sum testing and Fisher exact test, respectively, using R. Fifty-two articles were included, amounting to 204 individuals (1 female). Mean age of VEXAS symptom onset was 67 ± 5 years (range: 46-87). Orbital inflammation was the most common ocular manifestation, comprising periorbital edema (n = 83, 40.7%), orbital myositis (n = 14, 6.9%), dacryoadenitis (n = 6, 2.9%), and orbital compartment syndrome (n = 1, 0.5%). Other features included episcleritis (n = 28, 13.7%), scleritis (n = 28, 13.7%), uveitis (n = 25, 12.3%), and retinal vasculitis (n = 2, 1%), among others. Visual acuity reporting was limited (n = 4, 2%). Meta-analysis was conducted on 32 articles (n = 48) with genotype and ocular feature data. The most commonly reported UBA1 mutation was the missense mutation p.Met41Thr (n = 24, 50%), followed by p.Met41Val (n = 17, 35%), p.Met41Leu (n = 4, 8%), and splice site mutations or deletions (n = 3, 6%). There was an association for more severe ophthalmic features in the splice site mutation group vs methionine 41 missense mutations (P = .04). The most commonly associated systemic features included dermatologic manifestations (n = 41, 85%), recurrent fever (n = 38, 79%), and pulmonary involvement (n = 30, 63). There is notable variation in the ophthalmic features of VEXAS. Ophthalmic review is advised for VEXAS patients who develop eye symptoms, given the risk of sight-threatening disease.

Introduction:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by a dysregulated immune response against self-antigen, leading to multi-organ involvement. Myositis, as an initial manifestation of SLE, is a rare clinical entity, particularly in newly diagnosed patients.Case Presentation:A 27-year-old male presented with massive head and neck swelling, initially suspected to be superior vena cava syndrome (SVCS). Other symptoms included non-scarring alopecia, prolonged fever, oral ulcers, a history of hyperpigmented skin lesions, and progressive lower extremity weakness with edema. Hematological findings revealed persistent pancytopenia (anemia, leukopenia, and thrombocytopenia). Laboratory investigations demonstrated elevated muscle injury markers, including aspartate aminotransferase predominance and elevated creatine kinase. Immunological analysis showed a negative antinuclear antibody by indirect immunofluorescence, high anti-dsDNA titers, and normal complement levels. Bone marrow biopsy revealed trilineage dysplasia with macrophage activation, suggesting underlying hematologic involvement. Contrast-enhanced head and neck computed tomography ruled out SVCS, showing only diffuse muscle and subcutaneous edema. Based on the constellation of clinical, hematological, and imaging findings, the patient was diagnosed with myositis-associated SLE. The therapeutic approach included total plasma exchange (TPE), high-dose corticosteroid pulse therapy, and immunosuppressive induction therapy. Within 1 month of hospitalization, the patient demonstrated significant clinical and laboratory improvement and was subsequently transitioned to maintenance therapy with hydroxychloroquine (200 mg once daily), methylprednisolone (8 mg daily in a tapering regimen), and mycophenolate mofetil (500 mg twice daily). The patient achieved a lupus low disease activity state at follow-up.Discussion:This case represents a unique presentation of head and neck myositis in a newly diagnosed SLE patient, a manifestation not previously described in the literature. While orbital myositis in SLE has been reported, extensive myositis involving the head and neck as an initial SLE manifestation remains undocumented. Combining TPE, high-dose corticosteroids, and immunosuppressants was critical in disease control. Early recognition and aggressive immunomodulatory therapy are essential in managing such rare and severe SLE presentations.Conclusion:This case highlights an uncommon initial manifestation of SLE, emphasizing the importance of early clinical suspicion, comprehensive immunological and hematological evaluation, and prompt intervention. A multimodal therapeutic approach, including steroid pulse therapy, induction immunosuppression, and TPE, can lead to favorable clinical outcomes in severe and atypical SLE presentations.

ABSTRACT Purpose To develop a deep learning model using orbital computed tomography (CT) imaging to accurately distinguish thyroid eye disease (TED) and orbital myositis, two conditions with overlapping clinical presentations Methods Retrospective, single-center cohort study spanning 12 years including normal controls, TED, and orbital myositis patients with orbital imaging and examination by an oculoplastic surgeon. A deep learning model employing a Visual Geometry Group-16 network was trained on various binary combinations of TED, orbital myositis, and controls using single slices of coronal orbital CT images Results A total of 1628 images from 192 patients (110 TED, 51 orbital myositis, 31 controls) were included. The primary model comparing orbital myositis and TED had accuracy of 98.4% and area under the receiver operating characteristic curve (AUC) of 0.999. In detecting orbital myositis, it had a sensitivity, specificity, and F1 score of 0.964, 0.994, and 0.984, respectively. Conclusions Deep learning models can differentiate TED and orbital myositis based on a single, coronal orbital CT image with high accuracy. Their ability to distinguish these conditions based not only on extraocular muscle enlargement but also other salient features suggests potential applications in diagnostics and treatment beyond these conditions.

Macrophage activation syndrome (MAS) with hypoplastic marrow is a rare but life-threatening presenting manifestation of systemic lupus erythematosus. Lupus orbitopathy is also very rarely reported in the literature. We hereby report for the first time a 19-year-old girl who had a unique combination of MAS, with hypoplastic marrow and orbitopathy as a presenting feature of lupus. We discussed in detail our patient's demographic characteristics, clinical features, treatment, and outcome. Our patient responded well to high-dose glucocorticoids along with 6 monthly doses of intravenous cyclophosphamide as per the modified National Institute of Health protocol followed by tacrolimus as maintenance treatment. Her prednisolone dose could be tapered to 7.5mg daily after 8 months of treatment. We made a search on PubMed and Scopus for a literature review. We obtained 15 cases of MAS as a presenting feature in lupus, of which four had associated hypoplastic marrow. Males and juvenile age groups were equally affected with MAS as a presenting feature of lupus. Most of the patients responded to high-dose glucocorticoids. Only one patient succumbed to MAS. We also reviewed 14 cases of lupus orbitopathy with their clinical, and imaging characteristics, treatment, and outcome. Females predominated in these cases with the mean age being 43.6years (SD ± 16.9years). Treatment with glucocorticoids and immunosuppressive agents lead to complete resolution in most patients. The diagnosis of MAS with orbitopathy as a presenting feature of lupus in a 19-year-old girl poses a diagnostic challenge and requires the prompt exclusion of the common mimickers before initiation of aggressive immunosuppression.

The purpose of this study is to characterize orbital inflammatory disease (OID) in a primarily Black patient population, examining their demographics, presentations, workup, treatment, and outcome. A retrospective study was performed from January 2005 to June 2022 at two academic institutions in Brooklyn, NY. Patients included met criteria for one of the following OID conditions: non-specific orbital inflammation; nonbacterial dacryoadenitis; Tolosa-Hunt; orbital myositis; definite, possible, or probable IgG4-related ophthalmic disease; and sclerosing orbital inflammation. Data reviewed included orbital inflammatory labs, imaging, pathology, and treatment. Treatment was considered successful if a patient had complete resolution of symptoms. Thirty-nine patients met criteria for this study. 35.9% were diagnosed with dacryoadenitis, 28.2% with NSOI, 12.8% with myositis, 5.1% with possible IgG-ROD, 7.7% with probable IgG4-ROD, 7.7% with Tolosa Hunt, and 2.6% with sclerosing OI. 91% were started on steroids; 12.8% required transition to steroid-sparing therapy. 85% had a successful outcome with a resolution of symptoms. This study characterizes OID in a Black patient population and compares it to prior studies done on OID. Research on underrepresented patient populations is needed to understand differences in disease presentation and improve patient outcomes.

Unbalanced diabetes leads to orbital myositis with oculomotor nerve palsy (ONP)

Purpose: Although upper eyelid retraction is commonly associated with thyroid eye disease, its etiology remains unclear. This study evaluated the clinical features and treatment outcomes of patients with upper eyelid retraction caused by idiopathic orbital myositis (IOM).Methods: We conducted a retrospective analysis of the medical records of patients who presented with unilateral upper eyelid retraction. IOM was diagnosed based on normal thyroid function tests (TFT), including thyroid-stimulating immunoglobulin (TSI). Orbital imaging demonstrated contrast-enhanced enlargement of the superior rectus-levator palpebrae superioris complex (SR-LC). Pre- and post-systemic steroid treatment, margin-reflex distance 1 (MRD1), MRD1 difference between affected and unaffected eyes, exophthalmos, and diplopia were assessed.Results: In total, five patients (male: 4, female: 1) with a median age of 36.4 years were diagnosed with IOM. Three patients presented with diplopia on upgaze and supraduction limitation. Orbital imaging revealed levator palpebrae superioris muscle enlargement with distinct borders and homogeneous contrast enhancement. All cases with superior rectus enlargement demonstrated tendon involvement. The median duration from symptom onset to treatment initiation was 2.2 months. Four patients received oral prednisolone, whereas one received intravenous methylprednisolone. Although no significant improvements were observed in MRD1, MRD1 difference, or exophthalmos post-treatment, diplopia resolved in all three patients.Conclusions: IOM can present with upper eyelid retraction, emphasizing the importance of differentiating it from thyroid eye disease. TFT, including TSIs, and orbital imaging are essential diagnostic tools. These findings indicate that systemic corticosteroids can effectively manage diplopia associated with IOM, emphasizing the potential benefit of early and aggressive treatment.

Abstract BACKGROUND Orbital myositis (OM) is a rare extraintestinal manifestation (EIM) of inflammatory bowel disease (IBD) in which inflamed extraocular muscles cause severe eye pain. With fewer than thirty cases of OM described in the literature, its etiology remains largely unknown. We thus describe eleven cases of OM in patients diagnosed with IBD or microscopic colitis as well as prevalent comorbidities observed within this group. METHODS Patients with known orbital myositis diagnosed by an ophthalmologist and documented IBD or microscopic colitis were included at this single center institution from 2012 to 2022. RESULTS Of the eleven patients included in this study, six patients (55%) had Crohn’s disease, three patients (27%) had ulcerative colitis, one (9%) had indeterminate colitis, and one (9%) had lymphocytic colitis. All eleven patients were Caucasian and seven (64%) were female. The median age at OM presentation was 54 [47.5-60] years. On presentation, nine patients (82%) reported eye pain, seven (64%) reported blurry vision, four (36%) had headaches with concurrent photophobia, three (27%) had eye swelling, and three (27%) had diplopia. Six patients (55%) had unilateral OM and five (45%) had bilateral disease. Six OM cases were confirmed with ultrasound, two with MRI and three with clinical diagnoses. The median age at IBD diagnosis was 43 [37-51.5] years. At the time of OM presentation, all patients were in clinical remission for their IBD (6/11 patients). Only five patients (45%) were on IBD medication at the time of diagnosis; three were on a biologic medication (infliximab, adalimumab, and ustekinumab), with the others on sulfasalazine and budesonide. Regarding EIMs, two patients (18%) had aphthous ulcers, and nine patients (82%) had a history of IBD-related arthropathy. There were no recorded instances of uveitis, or dermatologic EIMs. Eight patients (72%) had a history of cervical (n = 7) or lumbar (n = 5) spinal disease requiring at least one neurosurgical intervention. Three patients (27%) had a positive ANA titer. Five patients (45%) were given steroids on presentation. Two patients (18%) were initially treated with NSAIDs. One patient (9%) was treated with bromfenac ophthalmic solution. Only three patients (27%) had documented OM flares after treatment; one was initially treated with NSAIDs and two with steroids. CONCLUSIONS Given its rarity, the true prevalence of OM is unknown. Our findings reaffirm prior studies’ observations about OM, including typical presentation and association with IBD. The number of patients in clinical remission for their IBD at the time of OM presentation may suggest a weaker correlation with underlying disease activity, in contrast to other EIMs. Additionally, the prevalence of cervical spinal disease among these patients, given their relatively younger age, warrants further investigation.

Abstract BACKGROUND Orbital myositis (OM) is a rare extraintestinal manifestation (EIM) of inflammatory bowel disease (IBD) in which inflamed extraocular muscles cause severe eye pain. With fewer than thirty cases of OM described in the literature, its etiology remains largely unknown. We thus describe eleven cases of OM in patients diagnosed with IBD or microscopic colitis as well as prevalent comorbidities observed within this group. METHODS Patients with known orbital myositis diagnosed by an ophthalmologist and documented IBD or microscopic colitis were included at this single center institution from 2012 to 2022. RESULTS Of the eleven patients included in this study, six patients (55%) had Crohn’s disease, three patients (27%) had ulcerative colitis, one (9%) had indeterminate colitis, and one (9%) had lymphocytic colitis. All eleven patients were Caucasian and seven (64%) were female. The median age at OM presentation was 54 [47.5-60] years. On presentation, nine patients (82%) reported eye pain, seven (64%) reported blurry vision, four (36%) had headaches with concurrent photophobia, three (27%) had eye swelling, and three (27%) had diplopia. Six patients (55%) had unilateral OM and five (45%) had bilateral disease. Six OM cases were confirmed with ultrasound, two with MRI and three with clinical diagnoses. The median age at IBD diagnosis was 43 [37-51.5] years. At the time of OM presentation, all patients were in clinical remission for their IBD (6/11 patients). Only five patients (45%) were on IBD medication at the time of diagnosis; three were on a biologic medication (infliximab, adalimumab, and ustekinumab), with the others on sulfasalazine and budesonide. Regarding EIMs, two patients (18%) had aphthous ulcers, and nine patients (82%) had a history of IBD-related arthropathy. There were no recorded instances of uveitis, or dermatologic EIMs. Eight patients (72%) had a history of cervical (n = 7) or lumbar (n = 5) spinal disease requiring at least one neurosurgical intervention. Three patients (27%) had a positive ANA titer. Five patients (45%) were given steroids on presentation. Two patients (18%) were initially treated with NSAIDs. One patient (9%) was treated with bromfenac ophthalmic solution. Only three patients (27%) had documented OM flares after treatment; one was initially treated with NSAIDs and two with steroids. CONCLUSIONS Given its rarity, the true prevalence of OM is unknown. Our findings reaffirm prior studies’ observations about OM, including typical presentation and association with IBD. The number of patients in clinical remission for their IBD at the time of OM presentation may suggest a weaker correlation with underlying disease activity, in contrast to other EIMs. Additionally, the prevalence of cervical spinal disease among these patients, given their relatively younger age, warrants further investigation.

Abstract Background Ocular extraintestinal manifestations (O-EIM) of inflammatory bowel disease (IBD) are rare, with most being attributed to scleritis, episcleritis and uveitis. Orbital myositis, however, is an exceedingly rare manifestation that is described as acute or chronic inflammation of one or more extraocular muscles leading to symptoms related to the mass effect including orbital pain, swelling, ophthalmoplegia, proptosis, and diplopia. To date, 3 reports of varying ocular myositis preceding IBD diagnosis have been described in pediatrics. Aims This report describes a case of unilateral orbital myositis, which was the initial and only symptom of newly diagnosed adolescent with Crohn’s disease (CD). Methods A chart review was conducted and placed into the context of this rare presentation. Results 15-year-old female presented with acute 6th cranial nerve palsy with ipsilateral optic disc edema, raising concern for raised intracranial pressure. Magnetic resonance imaging (MRI) revealed a retro-orbital mass on the left lateral rectus muscle, consistent with orbital pseudo tumor and myositis, no intracranial tumor or vascular lesion. She was treated with 3-month course of prednisone. An inflammatory condition was suspected based on the unusual presentation of orbital myositis which responded well to prednisone. Alternative infectious or rheumatologic causes of orbital pseudotumor were excluded and the patient was treated with corticosteroids prior to gastroenterology (GI) consultation. She was found to have elevated C-reactive protein (CRP), hypoalbuminemia and anemia at presentation. Fecal calprotectin (649)) was subsequently found to be positive at GI consultation after corticosteroid treatment initiation. Given the patient did not exhibit any GI signs or symptoms at the time of the evaluation, further GI investigations were deferred to after completion of steroids. Fecal calprotectin was persistently elevated (1463) after resolution of ocular symptoms and completion of steroids. Magnetic resonance enterography (MRE) identified inflammation of the terminal ileum. A definitive diagnosis of CD was established following upper GI endoscopy and colonoscopy with biopsies. Conclusions Orbital myositis is a rare ocular manifestation of CD. Early recognition and treatment are critical as they may precede the development of overt gastrointestinal symptoms, potentially delaying diagnosis if not considered. Given the rarity of this presentation, further studies are needed to better understand the pathophysiological link between CD and orbital myositis. Funding Agencies None