Orbital myositis induced by alendronate: A case report.

BACKGROUND AND PURPOSE: Bisphosphonates are widely used, notably for osteoporosis treatment. Their common side effects are well known. However, they can trigger less common effects such as orbital inflammation. Here, the case is reported of an orbital myositis triggered by alendronate. METHODS: This is a case report at an academic medical center. An orbital magnetic resonance imaging scan, a thoraco-abdominal computed tomography scan and blood sample analyses were performed. RESULTS: A 66-year-old woman treated by alendronate for her osteoporosis was investigated. She developed an orbital myositis after the first intake. Neurological examination revealed a painful diplopia with decreased downward and adduction movements of the right eye and edema of the upper eyelid. Orbital magnetic resonance imaging showed an orbital myositis of the right eye. No other cause of orbital myositis was found than the alendronate intake. After alendronate arrest and a short course of prednisone, the symptoms resolved. CONCLUSION: This case highlights that alendronate can cause an orbital myositis whose early diagnosis is of major importance because it is a treatable side effect.

Acute Bilateral Orbital Myositis Following Covid 19 Vaccination.

The authors present a case of acute bilateral orbital myositis occurring 24 hours after the administration of the mRNA1273 vaccination for COVID 19. The patient was presented with right proptosis, with orbital imaging demonstrating bilateral enlargement of all the extraocular muscles. Serological investigation did not reveal a precipitating cause or underlying disease process. The presenting features resolved entirely following treatment with methylprednisolone and the patient remains asymptomatic.

Bilateral orbital myositis in a patient with rheumatoid arthritis on treatment with tofacitinib: a report and review of literature.

Orbital myositis is a rare inflammatory condition affecting the extraocular muscles of the eyes. It has also been linked to systemic autoimmune diseases. We present a case of orbital myositis in a 57-year-old male undergoing treatment for rheumatoid arthritis (RA) with tofacitinib, a Janus kinase inhibitor (JAK). Prompt administration of intravenous steroids led to rapid symptom improvement. To date, only six published cases have documented the association between RA and orbital myositis. This is the first description of orbital myositis occurring during treatment with the anti-inflammatory drug tofacitinib, an increasingly used disease-modifying anti-rheumatic drug (DMARD). We review the literature and emphasize the importance of ongoing vigilance regarding adverse events linked to tofacitinib.

Idiopathic Orbital Myositis Revisited.

PURPOSE OF REVIEW: To review current knowledge regarding idiopathic orbital myositis. RECENT FINDINGS: 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.

Orbital myositis in systemic lupus erythematosus: a case-based review.

Ocular complications occur in up to one-third of patients with systemic lupus erythematosus (SLE). Among them, orbital myositis (OM) is considered a rare manifestation that affects the extraocular muscles and causes pain and restriction with eye movement. We report a case of OM in a 48-year-old female with SLE and secondary Sjogren's Syndrome, who presented headache, periorbital edema, and painful ocular movements in both eyes, with no other systemic manifestations. An orbital magnetic resonance image revealed thickening of the right medial rectus and left lateral rectus muscles. Laboratory tests were normal and there was no further disease activity. The patient was treated with prednisone 1 mg/Kg/day with a resolution of symptoms. We found 13 additional cases of OM from our literature review (11 SLE patients and 2 with discoid lupus erythematosus). There was a female predominance in these cases with a mean age of 43.6 years (SD ± 16.9). Their main clinical features included eye pain, swelling, proptosis, diplopia, and limitations in extraocular muscles, while in most of them, there was no other active systemic manifestation. Treatment with steroids led to the complete resolution of symptoms in most of these patients. The available evidence suggests that it is essential to have a high index of suspicion for OM in SLE patients even when there is no systemic disease activity so that proper treatment is initiated early.

Orbital giant cell myositis is an unusual and potentially lethal cause of bilateral ophthalmoplegia - A case report and literature review.

We present a case of orbital giant cell myositis (OGCM), presenting with bilateral subacute progressive ophthalmoplegia and optic nerve dysfunction. An early extraocular muscle biopsy confirmed the diagnosis and guided appropriate management. Comprehensive investigation excluded any underlying systemic disease, including myocarditis. Twenty two months after presentation, the patient remains well on azathioprine with complete resolution of orbital signs.

Orbital inflammatory disorders: new knowledge, future challenges.

PURPOSE OF REVIEW: This review aims to bring together recent advances in basic, translational and clinical research on the pathogenesis and treatment of orbital inflammatory conditions. RECENT FINDINGS: Basic science studies provide mechanistic insights into why the orbit is targeted for inflammation by autoimmune inflammatory disorders. Using Graves' disease as a test case reveals that endocrine pathways, such as the TSH and IGF1 receptor pathways play important roles in stimulating orbital inflammation. Furthermore, orbital tissues contain high concentrations of retinoids - byproducts of the visual pathway that diffuse across the sclera and can activate de novo transcription of inflammatory cytokines. Such cytokine expression places the orbit in a hyper-inflammatory 'resting' state, prone to respond to any additional systemic or local pro-inflammatory signals. The HIF2A--LOX pathway appears important for orbital tissue fibrosis. Lastly, bench-to-bedside studies of the IGF1R pathway have led to an FDA-approved drug, teprotumumab that represents a novel treatment approach for Graves' orbitopathy. Unfortunately, high drug costs and misplaced insurance company 'step-therapy' policies may block patients from receiving therapy that can protect vision and improve quality of life. SUMMARY: Improved understanding of orbital inflammatory conditions has led to a new drug and promises additional breakthroughs. Translational research is successful, but requires time, resources, and patience.

Atypical Cogan Syndrome Featuring Orbital Myositis and Dacryoadenitis.

A 39-year-old male presented with bilateral hearing loss and progressive left eye vision loss over a 14-month period. The development of systemic symptoms including arthralgias, enlarged lymph nodes, and profound leg weakness, prompted a workup for lymphoproliferative disease, infection, and autoimmune inflammatory conditions which was unrevealing. Subsequently, the right visual acuity declined from 20/25 to 20/70 and the left to hand motions due to corneal interstitial keratitis. There was limitation of left infraduction. Neuroimaging revealed dural thickening of the internal auditory canals, cavernous sinuses, cerebellum, and along the optic nerves. There was fusiform enhancing enlargement of the left inferior and medial rectus muscles and pathologic enlargement of the left lacrimal gland. Biopsy of the left lacrimal gland and left inferior rectus revealed fibrosis and lymphocytic infiltration. The patient was diagnosed with atypical Cogan syndrome and treated with oral prednisone, with improvement in visual acuity of the right eye, motility of the left eye, and systemic weakness.

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.

Granulomatosis with polyangiitis presenting as recurrent, multifocal orbital myositis.

A 43-year-old woman was referred with a 10 month history of persistent pain in the left orbit. Two years prior, she experienced similar pain in the right orbit. Magnetic resonance imaging (MRI) at the time revealed an enlarged right medial rectus muscle. She was diagnosed with idiopathic orbital myositis and was successfully treated with oral corticosteroids. A year later, she developed symptoms in the left orbit with similar imaging findings. For ten months, she remained on high dose corticosteroids for presumed left medial rectus myositis before presenting to our service. Computed tomography (CT) imaging after corticosteroid taper revealed enlarged left medial rectus and left lateral rectus muscles. Orbital biopsy established a diagnosis of granulomatosis with polyangiitis (GPA), for which she was successfully treated with rituximab. This case underscores the importance of not only proceeding with biopsy in atypical cases of orbital myositis but to also taper steroids prior to biopsy.

Suspected orbital myositis associated with COVID-19.

A 44-year-old male patient developed proptosis, edema, and erythema progressing to complete ptosis and supraduction deficit 2 days after positive COVID-19 test. He failed to improve on systemic antibiotics. MRI showed thickening and T2 enhancement of the superior rectus/levator complex consistent with orbital myositis. He improved on intravenous corticosteroids and experienced continued gradual improvement on oral steroids.

Orbital Myositis: A Comprehensive Review and Reclassification.

PURPOSE: 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. METHODS: A comprehensive literature review of orbital myositis was performed. RESULTS: 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. CONCLUSIONS: 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.

[Orbital myositis, a cause of pediatric painful ophthalmoplegia]. / Miositis orbitaria, una causa de oftalmoplejia dolorosa en pediatría.

INTRODUCTION: Orbital myositis (OM) is a serious inflammation of extraocular muscles with unknown etiology. Pe diatric presentation is rare and often affects more than one individual in a family, suggesting a genetic predisposition. OBJECTIVE: To describe a pediatric case of orbital myositis, its clinical characteristics, and the usefulness of MRI for confirming the diagnosis. CLINICAL CASE: A 13-year-old female patient presenting with acute headache, right periorbital pain, exacerbated by eye movements, and blurred vision. We ruled out thyrotoxic myopathy, infectious diseases, autoimmunity, and malignancy. An MRI showed right medial rectus muscle myositis and no evidence of optic neuritis. She was treated with intravenous systemic glucocorticoids followed by oral steroids with complete clinical resolution. CONCLUSIONS: OM has unknown etiology and can present a malignant course. Due to its unspecific clinical presentation, a comprehensive differential diagnosis should be made and it should consider performing MRI. Early treatment avoids permanent damage of extraocular muscles.

Case Series: Herpes Zoster Ophthalmicus with Acute Orbital Inflammation.

SIGNIFICANCE: Herpes zoster ophthalmicus (HZO) has variable initial manifestations, and acute orbital inflammation may be the first sign without apparent zoster rash. This case series is significant for presenting diverse clinical features and treatment options of HZO with acute orbital inflammation. PURPOSE: To report a case series of patients diagnosed as HZO with acute orbital inflammation including two cases with unique presentations. CASE REPORTS: Medical records of four patients of HZO with acute orbital inflammation were reviewed. Two men and two women with a median age of 57 years (range, 32 to 69 years) were diagnosed as having HZO with acute orbital inflammation. Initial presentations included two cases of zoster rash and two cases of orbital pain preceding vesicles. Clinical orbital findings included proptosis, ptosis, ophthalmoplegia, and decreased visual acuity. Orbital magnetic resonance image showed enlarged extraocular muscle with enhancement and optic nerve sheath enhancement in all four patients, and unilateral dacryoadenitis in one patient. All four patients were administered with systemic steroid, three patients received intravenous acyclovir, and one patient received oral acyclovir. Orbital signs improved in all patients over several months. CONCLUSIONS: Herpes zoster ophthalmicus may initially present with orbital inflammatory signs, such as acute orbital myositis, perioptic neuritis, or dacryoadenitis, without zoster rash. Physicians should be aware of acute orbital inflammation as a presenting sign of HZO.

Unilateral Migratory Relapsing Orbital Myositis While on Immunosuppressant Therapy: An Uncommon Entity.

A young healthy female presented with acute onset left ocular pain, restricted ocular motility, and binocular diplopia. CT imaging showed left lateral rectus myositis that resolved with oral corticosteroids. Two sequential relapses occurred subsequently involving the superior rectus-levator complex followed by the medial rectus. Biopsy revealed orbital inflammatory disease with lymphocytic vasculitis. Detailed systemic work up was normal. The second relapse was seen while on long-term oral methotrexate although initial disease remission had been achieved with the same drug. A changeover to oral azathioprine was able to achieve disease remission after the second relapse. All relapses involved the same side and the contralateral orbit was not affected. This report presents the curious phenomenon of unilateral migratory relapsing orbital myositis of unknown cause that recurred even while on immunosuppressant therapy. It highlights the unpredictable nature of this uncommon entity and the challenges faced in managing such cases.

Dacryoadenitis and extraocular muscle inflammation associated with contact lens-related Acanthamoeba keratitis: A case report and review of the literature.

The present report discusses a new case of dacryoadenitis with extraocular muscle inflammation associated with Acanthamoeba keratitis (AK) in a contact lens wearer. A 41-year-old male, who has worn silicone hydrogel contact lenses on an extended basis for about 10 years, attended with the complaints of vision disturbance, hyperemia, and pain in his right eye. His history revealed that 1.5 month ago, he had been diagnosed with allergic conjunctivitis and had used steroid eye drops. Biomicroscopic examination revealed eyelid edema, chemosis, and ring infiltration, radial keratoneuritis and an epithelial defect in the cornea. Magnetic resonance imaging demonstrated enlarged lacrimal gland with edematous changes consistent with inflammation due to dacryoadenitis. There were also thickening and edema of the right superior oblique and lateral rectus muscle. The treatment protocol for AK was applied with no specific treatment for dacryoadenitis. After 4 months of the treatment, dacryoadenitis and keratitis regressed. Dacryoadenitis and extraocular muscle inflammation may accompany AK more frequently than expected and previously known. The evaluation of the lacrimal gland and extraocular muscles in presence of AK might be beneficial for understanding better the exact clinical picture and course of the keratitis.

Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review.

BACKGROUND: Involvement of orbital structures by immunoglobulin G4-related disease (IgG4-RD) is not uncommon. We conducted this study to evaluate the clinicopathologic features of orbital IgG4-RD. MATERIAL/METHODS: This was a retrospective, clinicopathologic study. Clinical records, light microscopic features, results of immunostaining with IgG & IgG4 and laboratory findings were reviewed in 16 patients diagnosed with orbital IgG4-RD. RESULTS: Eleven patients had a bilateral disease, and the lacrimal gland was involved in 14. Dense sclerosis, plasma cell aggregates and dense lymphoplasmacytic infiltrate were seen in all patients. Serum IgG4 titre was elevated in 12 patients. Nine patients responded completely to glucocorticoid treatment. Five patients had a relapse on discontinuation of treatment. CONCLUSION: Orbital IgG4-RD is a distinct clinicopathologic entity requiring increased awareness and needs to be differentiated from other orbital lymphoproliferative lesions.

Lateral rectus myositis mimicking an abducens nerve palsy in a pregnant woman.

Myositis is a rare unknown inflammatory disorder of the skeletal muscle tissue. Generalized inflammatory myopathies, polymyositis, and dermatomyositis have been reported during pregnancy. Isolated orbital myositis in pregnancy has not been previously described in the literature. The authors report a case of left isolated orbital myositis in a primigravida at 38 weeks gestation affecting the patient's left lateral rectus muscle. MRI of the orbit was consistent with the diagnosis. She showed remarkable clinical improvement with oral corticosteroids therapy.

Adalimumab as steroid-sparing treatment of inflammatory-stage thyroid eye disease.

PURPOSE: Steroids are often used as medical therapy for active thyroid eye disease (TED). While high-dose steroids have been shown to be effective in reducing the severity of TED symptoms, the side effects of steroids can be severe. As the pathogenesis of TED is thought to involve the upregulation of proinflammatory cytokines, including tumor necrosis factor-α (TNF-α), it has been postulated that anti-TNF agents may be used as steroid-sparing agents in the treatment of TED. This retrospective study was conducted to examine the efficacy of adalimumab, a subcutaneously administered TNF-α antagonist, in treating the inflammatory symptoms of active TED. METHODS: All patients in the inflammatory phase of TED who were treated with adalimumab at the Jules Stein Eye Institute over a 2-year period were reviewed. Data concerning visual acuity, optic nerve function, extraocular motility restriction, binocular visual fields, and proptosis were extracted from patient charts. Clinical photographs from baseline and 3-month follow-up visits were reviewed by masked orbital specialists. Each photograph was graded on the severity of conjunctival injection, chemosis, eyelid erythema, and eyelid edema on a scale from 1 to 4. An inflammatory score was calculated as the sum of these 4 elements. Groups were compared using paired t tests. RESULTS: Six of 10 patients showed a decrease in inflammatory score while on adalimumab, whereas 3 showed an increase and 1 stayed the same. One patient experienced a significant complication (hospital admission for sepsis). Eight patients received concomitant tapering steroids during the first 6 weeks of therapy as the adalimumab reached maximum efficacy. When data from all 10 subjects were analyzed together, there was no significant change in inflammatory index after 3 months of treatment with adalimumab. However, when the 5 patients with a high baseline inflammatory index (>4) were considered separately, there was a significant improvement (mean decrease of 5.2±2.7; p<0.01) after adalimumab treatment. Four of 5 patients also reported a subjective improvement in symptoms while on adalimumab. CONCLUSIONS: This study suggests that adalimumab may have a role in the treatment of active TED with prominent inflammatory symptoms. The use of adalimumab and other immunosuppressive agents in the treatment of TED may help to mitigate some of the metabolic and psychiatric side effects of pulsed steroid treatment. A future randomized controlled study will be necessary to determine the efficacy of adalimumab as a primary therapy for TED.

Idiopathic orbital myositis.

PURPOSE: There is a paucity of reliable data and limited experience on the clinical features and therapeutic outcomes of orbital myositis. The purpose of this study was to collate data on the clinical features, imaging, diagnosis, and therapeutic effects of case reports from patients with myositis of idiopathic orbital inflammation pseudotumor in a tertiary eye hospital in China. METHODS: A retrospective study was performed on the records of 44 Chinese patients with orbital myositis. Data were obtained from the period of January 1, 2000, to August 31, 2010, from patients treated at the Eye Hospital of the Zhongshan Ophthalmic Center at Sun Yat-sen University, Guangzhou, China. RESULTS: Twenty-five patients were women and 19 men. The mean age was 39.1 years (range, 11-77 years). Right eyes were involved in 18 cases, left in 17 cases, and both eyes in 9 cases. Dysfunctions of the affected muscles included pure paretic (20.5%), pure restrictive (45.5%), or mixed paretic-restrictive (34.1%). The ratio of acute to subacute stage was 1:3. The rank order of affected muscles was as follows: superior rectus (29.1%), lateral rectus (25.6%), medial rectus (24.4%), inferior rectus (19.8%), and superior oblique (1.16%). The proportion of single muscle involvement was 37.5%, and tendon involvement was seen in 40.9% of the muscles. All patients were treated with systemic corticosteroids (prednisone or dexamethasone). Full recovery was achieved in 38.6% of patients, whereas 59.1% achieved partial recovery with an average of 6.4 recurrences (range, 2.0-8.0 recurrences). Recurrences occurred in 81.8% of the patients. CONCLUSIONS: Orbital myositis occurs in multiple clinical manifestations and may be recurrent. Imaging is an important technique for use in diagnosis. Systemic corticosteroid represents an effective approach for treatment.