Idiopathic Orbital Myositis Research Articles

Treatment Outcomes of Patients with Orbital Inflammatory Diseases: Should Steroids Still Be the First Choice?

Objective: To clarify the therapy response in orbital inflammatory diseases (OID), we analyzed the treatment effects of steroid therapy, the use of disease-modifying antirheumatic drugs (DMARDS), and biologicals in our tertiary referral center cohort. Methods: We collected the clinical and demographic data of all patients treated for non-specific orbital inflammation (NSOI) (n = 111) and IgG4-ROD (n = 13), respectively at our center from 2008 to 2020 and analyzed them with descriptive statistics. NSOI were sub-grouped according to the location into either idiopathic dacryoadenitis (DAs) (n = 78) or typical idiopathic orbital myositis (n = 32). Results: Mean age at first clinical manifestation was significantly different between subgroups (IOI: 49.5 ± 18, IgG4-ROD: 63.2 ± 14, p = 0.0171). Among all examined OID, 63 patients (50%) achieved full remission (FR) with corticosteroids (NSOI 53%/IgG4-ROD 31%). In contrast, classic myositis showed a significantly higher response (76%). Disease-modifying drugs (DMARDS) for myositis accomplished only 33% FR (NSOI 57%) and 66% did not respond sufficiently (NSOI 43%). The biologic agent (Rituximab) was significantly more efficient: 19 of 23 patients (82%) achieved full remission and only 4 (17%) did not respond fully and needed orbital irradiation or orbital decompressive surgery.

Magnetic Resonance Imaging of Idiopathic Orbital Myositis.

To characterize the qualitative and quantitative features of idiopathic orbital myositis (IOM) on MRI. This was a multicenter retrospective study of patients with active IOM with MRI. Patients with incomplete clinical records, poor-quality or interval scans without active myositis, and specific orbital myositis (i.e., orbital myositis secondary to an identified condition) were excluded. An enlargement ratio was calculated by dividing the diameters of the affected extraocular muscle (EOM) by the contralateral unaffected EOM. Twenty-four patients (mean age: 44.4 ± 17.8 years-old, male: 11) between 2011 and 2022 were included. One case (4.2%) was pediatric (17 years old), and 6 cases presented with recurrence. Active IOM was characterized by fusiform EOM enlargement, high T2 signal, and contrast enhancement. Average maximal EOM diameters ranged from 4.6 to 7.7 mm (enlargement ratio: 1.4-2.2). Eighteen (75%) patients had single EOM involvement, most commonly the medial rectus. Other ipsilateral structures affected included focal orbital fat (16/24, 66.7%) and lacrimal gland (8/24, 33.3%). Contralateral changes in the EOM and/or lacrimal gland were observed in 7 patients (29.2%). Patients presenting with recurrence were likely to develop ongoing recurrent episodes ( p = 0.003). Various radiological patterns of involvement described including EOM enlargement, contrast enhancement, abnormal signal, and involvement of other orbital structures are indicative of active IOM. IOM remains a heterogeneous spectrum of acute and chronic clinico-radiological presentations. Inflammation may involve other ipsilateral or contralateral orbital structures or may be bilateral despite presenting clinically as unilateral disease. Quantitative measurements may have utility in differentiating IOM from other causes of orbital myositis.

A peculiar case of idiopathic orbital myositis in adolescence

Abstract
 Introduction : Idiopathic orbital myositis, an inflammation localized at the extraocular muscles, is rarely reported in pediatrics. Until now, the prevalence and incidence of orbital myositis remain unknown. This paper aims to report a case of idiopathic orbital myositis in a young girl with headache.
 Case Illustration : A-16-year-old presented with a droopy eyelid of the left eye (LE) and double vision since 1 week before admission. She also experienced swollen eyelid and cluster headache for 5 months with no neurological abnormalities. Ophthalmic examinations showed exotropia with limited eye movement of LE. Ptosis of LE was noted with margin reflex distance 1 (MRD-1) +2 mm, while MRD-1 +6 mm for the right eye. Other examinations were unremarkable with normal funduscopy and negative RAPD. Non-contrast orbital sinus CT-Scan revealed an enlargement of the superior rectus and lateral rectus muscle. The laboratory result showed slight leucocytosis and normal ESR. High-dose intravenous corticosteroid was administered and tapered with oral steroid. The orbital clinical findings showed improvements, better ocular movements, and diminished headache complaints.
 Discussion : As a rare case, the diagnosis of orbital myositis in adolescence can be challenging and requires detailed examination, and proper imaging to exclude other diagnosis such as ophthalmoplegic migraine, infections, malignancies, or congenital lesions (hemangioma). The first-line therapy is a systemic corticosteroid, which well responded in this patient.
 Conclusion : In this patient, intravenous corticosteroid represent an effective treatment. Close monitoring of the disease progression is required after switching to oral therapy to prevent relapse.

Isolated Lateral Rectus Myositis – A Case Series

Idiopathic orbital myositis is a rare, nongranulomatous, noninfective, nonneoplastic inflammation of the orbit. It can involve either a single extraocular muscle or multiple extraocular muscles, unilaterally or bilaterally. Isolated lateral rectus myositis is less commonly described. It can be diagnosed clinically or radiologically. The characteristic clinical features include painful eye movements, periorbital edema, proptosis, and diplopia. The typical features on magnetic resonance imaging include fusiform enlargement of the extraocular muscles, with enhancement on contrast. Treatment with corticosteroid results in prompt improvement in symptoms and remission. We hereby report two patients with isolated, acute, unilateral lateral rectus myositis.

Idiopathic Orbital Myositis Revisited.

To review current knowledge regarding idiopathic orbital myositis. Recent publications have focused on possible causes of orbital myositis and the process to reach a diagnosis of idiopathic orbital myositis. With inflamed and enlarged extraocular muscles, features to distinguish between competing diagnostic possibilities are based on imaging in the context of history and clinical signs. Idiopathic orbital myositis is characterized by the clinical triad of acute onset of orbital pain exacerbated on eye movement, double vision, and redness or swelling of the eyelids or conjunctiva, along with the radiological finding of homogeneous, fusiform enlargement of one or more extraocular muscles. In atypical or inconclusive clinico-radiological findings for a diagnosis of idiopathic orbital myositis, or where the clinical behavior changes or fails to respond to corticosteroid treatment, a systemic and oncologic work-up and muscle biopsy are warranted to exclude specific local or systemic disease as cause of the inflamed and enlarged muscle. As our understanding of idiopathic orbital myositis evolves, the diagnostic focus is shifting toward earlier identification of underlying local or systemic disease through systemic work-up and muscle biopsy.

CASE REPORT OF IDIOPATHIC BILATERAL ORBITAL MYOSITIS

Orbital myositis is an inflammatory disease affecting extraocular muscles. Mostly unilateral and rarely bilateral orbital involvement is seen. Bilateral involvement is a secondary table to systemic diseases and recurrence can be seen. Diagnosis is made by clinical, examination and imaging. Increased density of extraocular muscles and increased muscle mass should be seen in computed tomography or magnetic resonance imaging. The basis of the diagnosis is exclusion and the differential diagnosis needs to be done well. Steroids and other immunosuppressive agents may be used in the treatment. Patients respond dramatically to steroid treatment. While short-term steroid treatment may be sufficient in idiopathic patients, orbital myositis secondary to systemic disease requires longer-term and non-steroid immunosuppressive treatments. Here, a case of idiopathic orbital myositis with bilateral involvement is presented as it is a rare condition.

Eye pain in 13-year-old teenager: a case report of idiopathic orbital myositis

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Myositis of an Extraocular Muscle, a Possible Drug Reaction: Histopathologic and Immunopathologic Analysis

A 58-year-old man presented with left-sided orbital inflammation, including chemosis and a lateral rectus abduction defect. Initially presumed to represent cellulitis, the condition responded poorly to oral and intravenous antibiotics. CT showed the epicenter of an infiltrate to involve the lateral rectus. The patient improved dramatically when oral prednisone was added. Lateral rectus biopsy displayed intramuscular polyclonal lymphoid infiltrates, rich with eosinophils. Complete resolution of the inflammatory process was confirmed by a follow-up CT. The presumptive diagnosis was idiopathic orbital myositis, an uncommon condition of unknown etiology. However, the patient had taken rosuvastatin, which has been rarely associated with diplopia and ophthalmoplegia, raising the question of whether this case was truly idiopathic.

Isolated Unilateral Ptosis Caused by Idiopathic Orbital Myositis

Idiopathic orbital myositis is considered as a subgroup of idiopathic orbital inflammatory disease. It is a non-infectious inflammatory disorder primarily affecting the extraocular muscles and causes various eye symptoms including pain, diplopia and limitation of extraocular movement. Cases of isolated ptosis by idiopathic orbital myositis have been very rarely described in the literature. We report a patient who developed unilateral painless ptosis caused by idiopathic orbital myositis. A 52-year-old man presented with drooping of the right eyelid for 3 days. There was no history of headache, double vision or any other complaints. Neurological examination revealed right ptosis without pupil and extraocular muscles involvement. Repetitive nerve stimulation test was normal. Ptosis did not improve after the neostigmine injection. Magnetic resonance imaging scan showed asymmetric enlargement of right superior rectus/levator palpebrae superioris muscle complex and medial rectus muscle. Ptosis resolved dramatically after oral corticosteroid therapy. Isolated unilateral ptosis can be caused by various etiologies. Idiopathic orbital myositis should be considered in the differential diagnosis of ptosis. Keywords: Orbital myositis, Blepharoptosis, Oculomotor muscles

Histopathologic Findings in Idiopathic Orbital Myositis

To report the histologic and clinical features of idiopathic orbital myositis (IOM) patients who underwent extraocular muscle (EOM) biopsy over 20 years, to provide the first methodical reference to the expected histopathologic findings, and to discuss the histopathologic differences from common differential diagnoses. Cohort study. All patients with a diagnosis of IOM who underwent EOM biopsy from 2000 through 2019 were included. Patients who had a different final diagnosis were excluded. Tissue samples of EOM and medical records of all participants were reviewed. Histopathologic features of muscle biopsy, including tissue morphologic features and cellular composition. Thirteen patients met both inclusion and exclusion criteria, and their tissue samples were revisited. Nine patients showed histopathologic findings that suggested a conclusive diagnosis of IOM, and the study focused on them. The average age at presentation was 49 years, and 66.7% of patients were women. The most commonly biopsied EOM was the medial rectus (44.4%). The most common indications for biopsy were nonresolving orbital disease with inadequate response to corticosteroids (44.4%) or a high suspicion of malignancy because of known pre-existing systemic malignancy or the presence of an atypical orbital mass in addition to enlarged muscles (44.4%). The histopathologic findings that suggested a diagnosis of IOM were splaying of muscle fibers by inflammatory infiltrates (n= 9) and mild fibrosis (n= 8) in the endomysium or replacing muscle fibers, with no granulomas or vasculitis. The inflammatory infiltrates identified were of chronic inflammatory cells, consisting of lymphocytes (n= 9), plasma cells (n= 6), and histiocytes (n= 6). Other less commonly identified cells were eosinophils (n= 4), polymorphonuclears (n= 1), and giant cells (n= 1). Muscle fiber degeneration or regeneration was evident in 5 patients. Four patients from the initial cohort showed inconclusive histologic findings on revision and were reassigned as suspected IOM. The histopathologic features of involved muscles in IOM resemble those seen in idiopathic orbital inflammation and differ from those seen in common differential diagnoses. Extraocular muscle biopsy should be strongly considered whenever the presentation of orbital myositis is not typical or when significant underlying conditions are a possibility.

Treatment of idiopathic orbital myositis with frequent relapses: First case with tacrolimus and review of literature

A 41-year-old man presented with a 2-year history of recurrent painful ophthalmoplegia. Previous brain magnetic resonance imaging showed isolated enlargement of extraocular muscles with enhancement. A diagnosis of idiopathic orbital myositis (IOM) was given. Though the patient initially responded dramatically to corticosteroid, he began developing steroid resistance from his third attack and experienced frequent relapses. Therefore, tacrolimus was added to his regimen. The patient had full symptom recovery after tacrolimus and reported no relapses in the 1-year follow-up. This is the first case showing the efficacy of tacrolimus in treatment of IOM. Our case illustrates the importance of immunosuppressive agents in IOM patient refractory to steroid for both symptom control and relapse prevention.

Clinical Features of Ocular Motility in Idiopathic Orbital Myositis.

Objective: To elucidate the clinical features of ocular motility and the risk factors for recurrence in idiopathic orbital myositis. Methods: The medical records of 31 patients diagnosed with idiopathic orbital inflammation between 2003 and 2019 were retrospectively reviewed. All patients were initially treated with corticosteroids. Treatment outcome and ocular motility were noted. Results: Twenty-six patients (84%) had unilateral involvement and five patients (16%) were bilateral. Of the 31 patients, 22 patients (71%) showed ocular motility limitation. The mean grading scale of extraocular muscle (EOM) limitation was −1.65 ± 1.80. EOM limitation was found in the same direction of the most affected muscle in 14 patients (64%), while 8 patients (36%) showed duction limitation in the opposite direction. Nine patients (35%) suffered from recurrence. Recurrence was more likely to occur in patients with multiple muscle involvement (p < 0.001). The interval to relapse of symptoms after discontinuation of steroids was significantly shorter in patients with multiple recurrences compared to those with a single recurrence (1.8 ± 0.8 weeks versus 6.0 ± 1.4 weeks, p = 0.020). Conclusions: Idiopathic orbital myositis showed variable degrees of ocular motility limitation, and limitation in the same direction of the action of the affected muscle was more frequent. Recurrent myositis was more likely to have multiple muscle involvement. Rapid relapse of symptoms after discontinuation of steroids was a significant indicator of multiple recurrences.

Orbital Myositis: A Comprehensive Review and Reclassification.

Orbital myositis occurs in typical and atypical forms. This review summarizes and updates the current state of knowledge of all forms of inflammation affecting extraocular muscle, excluding thyroid-associated orbitopathy. A comprehensive literature review of orbital myositis was performed. Orbital myositis typically occurs in an idiopathic acute form, with painful diplopia due to inflammation in 1 or more extraocular muscles of young adult females, which usually responds to a course of oral corticosteroids. Atypical forms include idiopathic chronic or recurrent orbital myositis, and myositis related to systemic autoimmune, inflammatory, and infective conditions. The commonest associated autoimmune condition is inflammatory bowel disease. Immunoglobulin G4-related ophthalmic disease often affects extraocular muscle. Drug reactions and rarely paraneoplastic disease may also cause extraocular muscle inflammation. Orbital myositis occurs in a typical acute steroid responsive form, but atypical forms related to specific autoimmune and inflammatory conditions are increasingly recognized. Orbital myositis has many similarities to uveitis and would benefit from a systematic approach to nomenclature, diagnosis, and treatment.

Recurrent Idiopathic Orbital Myositis: Two Case Reports

Idiopathic orbital myositis is a subtype of idiopathic orbital inflammatory diseases characterized by inflammation of the extraocular muscles. The diagnosis of the disease is made clinically and radiologically. Clinically characterized by periorbital edema, ptosis, propitosis, diplopia and painful eye movements. The diagnosis is made by observing the enlargement of the involved muscles in the orbital CT. The most common differential diagnosis is thyroid ophthalmopathy. In this study, we presented two patients with idiopathic orbital myositis who were referred to our clinic with complaints of eye rash, eye movement pain, bilateral visual acuity. Idiopathic orbital myositis was diagnosed and 1 mg/kg/day oral prednisolone was started. Symptoms of the patients regressed with oral corticosteroid therapy.

A Rare Cause of Diplopia: Idiopathic Orbital Myositis; A Case Report and Review of the Literature

A Rare Cause of Diplopia: Idiopathic Orbital Myositis; A Case Report and Review of the Literature

Long-term Outcome in 7 Patients With Idiopathic Orbital Myositis.

Purpose:Idiopathic orbital myositis, a rare disease of unknown cause, presents a diagnostic puzzle because the diagnosis is based on the exclusion of other diseases. This study aims at elucidating its long-term outcome to answer a clinical question whether idiopathic orbital myositis would be a distinct clinical entity.Methods:Retrospective review was made on 7 consecutive patients (6 men and 1 woman) with the age at the initial visit ranging from 33 to 69 years (mean, 45.8 years) who were diagnosed with idiopathic orbital myositis and followed for 5 years or more (mean, 9.2 years) at a referral-based hospital.Results:Chief complaint at the initial visit was diplopia in 4 patients, blurred vision in 2 patients, and proptosis in 1 patient. On magnetic resonance imaging, 4 patients showed enlargement of a single extraocular muscle on unilateral side while 3 patients showed enlargement of multiple extraocular muscles on unilateral side or bilateral sides. No patient developed systemic diseases or other orbital lesions in the long-term follow-up. All patients at the last visit were free from symptoms, including diplopia, after tapering of prednisolone at the initial dose of 20 to 60 mg daily.Conclusion:Idiopathic orbital myositis had a favorable long-term outcome with corticosteroid administration and appears to be a distinct clinical entity without systemic involvement.

Idiopathic Orbital Myositis with Pseudosixth Nerve Palsy: The Importance of Neuroimaging

Idiopathic Orbital Myositis with Pseudosixth Nerve Palsy: The Importance of Neuroimaging

Consensus on Diagnostic Criteria of Idiopathic Orbital Inflammation Using a Modified Delphi Approach

Current practice to diagnose idiopathic orbital inflammation (IOI) is inconsistent, leading to frequent misdiagnosis of other orbital entities, including cancer. By specifying criteria, diagnosis of orbital inflammation will be improved. To define a set of criteria specific for the diagnosis of IOI. A 3-round modified Delphi process with an expert panel was conducted from June 8, 2015, to January 25, 2016. Fifty-three orbital scientist experts, identified through membership in the Orbital Society, were invited to participate in on online survey and they scored, using 5-point Likert scales, items that are eligible as diagnostic criteria from the literature and from personal experience. The items were clustered around the anatomic subtypes of IOI: idiopathic dacryoadenitis and idiopathic orbital fat inflammation (2 nonmyositic IOIs), and idiopathic orbital myositis (myositic IOI). Items with dissensus were rescored in the second round, and all items with consensus (median, ≥4; interquartile range, ≤1) were ranked by importance in the third round. Consensus on items to be included in the criteria. Of the 53 experts invited to participate, a multinational panel of 35 (66%) individuals with a mean (SD) years of experience of 31 (11) years were included. Consensus was achieved on 7 of 14 clinical and radiologic items and 5 of 7 pathologic items related to diagnosis of nonmyositic IOI, and 11 of 14 clinical and radiologic items and 1 of 5 pathologic items for myositic IOI. There was agreement among panelists to focus on surgical tissue biopsy results in the diagnosis of nonmyositic IOI and on a trial with systemic corticosteroids in myositic IOI. Panelists agreed that a maximum number of 30 IgG4-positive plasma cells per high-power field in the orbital tissue is compatible with the diagnosis of IOI. An international panel of experts endorsed consensus diagnostic criteria of IOI. These criteria define a level of exclusion suggested for diagnosis and include tissue biopsy for lesions not confined to the extraocular muscles. This consensus is a step toward developing guidelines for the management of IOI, which needs to be followed by validation studies of the criteria.

Idiopathic orbital myositis in a pediatric patient and treatment

Idiopathic orbital myositis in a pediatric patient and treatment

Idiopathic orbital myositis presenting as cluster headache: a case report

Idiopathic orbital myositis presenting as cluster headache: a case report