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.

Rapid Loss and Recovery of Vision Following Steroid Treatment in Orbital Myositis: A Case Report.

A 65-year-old African American man initially presented to the emergency department complaining of headaches, retro-orbital pressure, decreased vision, white flashes and floaters, and palinopsia of both eyes. After complete evaluation, he was diagnosed with migraine with aura and discharged to home with an ophthalmology follow-up. Upon follow-up with the ophthalmology team, he had developed severe periorbital inflammation, proptosis, chemosis, and vision loss that was greatest on the left side. The patient was immediately hospitalized for further evaluation and steroid treatment. His vision, ocular symptoms, and physical findings dramatically and rapidly improved with a 3-day course of high-dose intravenous steroids. Existing literature is sparse on rapid loss and recovery of vision following steroid treatment for orbital myositis. The exact mechanism of vision loss in orbital myositis is not understood and merits further investigation. Orbital myositis is a subset of nonspecific orbital inflammatory syndrome. It remains a poorly understood condition that mimics other, more common conditions such as thyroid eye disease and orbital cellulitis. If left untreated, orbital myositis could progress to the point of continued inflammation, enlargement of ocular tissues, ocular ischemia, and optic neuritis. To reverse these symptoms and prevent further progression, a quick diagnosis followed by steroid treatment is imperative.

Efficacy of Infliximab in Disease Control of Refractory Orbital Myositis.

PURPOSE: Orbital myositis is a common cause of orbital inflammation with localized involvement of the extra ocular muscles. This study aimed to assess the value of infliximab in controlling orbital myositis and reducing its relapse rate. METHODS: We conducted a retrospective review of the medical records of all consecutive patients with orbital myositis treated with infliximab between 2007 and 2016. We examined change in corticosteroid and immunomodulatory doses as well as relapse rates following treatment with infliximab. RESULTS: The study included seven patients with an average follow up of 19 ± 13.4 months. The mean dose of systemic corticosteroid was reduced from 28.57 ± 14.35 mg/day at the time of infliximab initiation to 7.00 ± 6.83 mg/day at final follow-up (p = .003). Long-term remission was achieved in 85.7% (n = 6). CONCLUSION: This study supports the role of infliximab in treating refractory orbital myositis and this was associated with clinical improvement, decreasing relapse rate with dose reduction of conventional treatment.

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.

Infliximab Reverses Symptoms and May Protect from Developing Chronic Restrictive Ophthalmopathy in Children with Familial Orbital Myositis: A Case Report.

BACKGROUND: Orbital myositis is a rare sporadic eye disease associated with extraocular eye muscle inflammation. To date, there have been two reports of familial orbital myositis (FOM), which demonstrate partially penetrant autosomal dominant inheritance. CASES: We report six new Australian cases of FOM, four of whom extend one of the reported pedigrees, as well as a separate mother and daughter manifesting orbital myositis, which constitutes a third report of familial occurrence. We can confirm that the disease has onset in childhood, appearing to go into remission in adult life, and that the inflammation is corticosteroid-responsive. However, one patient went on to develop permanent diplopia in upgaze. We also report two children suffering chronic pain and diplopia who demonstrated complete resolution of symptoms with the anti-TNF-α monoclonal infliximab. CONCLUSION: Uncontrolled FOM in childhood may result in permanent extraocular eye muscle damage, while TNF-α blockade provides an excellent steroid-sparing effect.

Orbital myositis and scleritis after anti-SARS-CoV-2 mRNA vaccines: A report of three cases.

PURPOSE: To report three cases of ocular myositis and scleritis, bilateral scleritis and unilateral single muscle myositis after mRNA COVID-19 vaccination. METHODS: Case series of three patients who presented to the Orbit Outpatient Service of Fondazione Policlinico Universitario A. Gemelli with a history of unilateral proptosis, diplopia and pain, bilateral red eye and pain during eye movements and unilateral proptosis and inconstant diplopia respectively with onset 5-10 days after m-RNA COVID-19 vaccine. A thorough hematologic work up and orbital contrast enhanced magnetic resonance imaging (MRI) in patients with proptosis was performed. RESULTS: Patients were females, 64, 58 and 45 years old respectively. MRI showed enlargement of all right rectus muscles, with both muscle belly and insertion involvement in the first case associated to right scleritis. A bilateral scleritis was diagnosed in the second patient and a single muscle myositis in the third patient. Serological tests excluded thyroid diseases. The first and second patient were treated respectively with oral and topical glucorticoids with a complete clinical response. Two 2 cycles of oral non-steroidal anti-inflammatory drugs were administered to the third patient with a partial response. CONCLUSION: As far as we know these are the first report of orbital myositis and scleritis presenting after mRNA BNT162b2 vaccine (Pfizer/BioNTech) and mRNA-1273-(Moderna) vaccine, an uncommon effect of a likely autoimmune reaction triggered by the virus antigen.

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.

A unique case of orbital inflammatory syndrome following COVID-19 infection.

Orbital inflammatory disease is a rarely reported complication of COVID-19 infection. We report a unique case of bilateral orbital myositis in a 12-year-old boy who tested positive for COVID-19 without typical systemic symptoms. Workup for other infectious and inflammatory etiologies was negative. After failing both oral and intravenous antibiotics, the patient was started on high-dose systemic steroids, with significant clinical improvement after 24 hours, thus confirming the inflammatory etiology of his presentation.

Diagnostics and treatment of orbital myositis.

Objective: Orbital myositis is a rare clinical condition that involves idiopathic inflammation mostly of extraocular muscles. The purpose of this study was to present a diagnostic and treatment plan of orbital myositis. Methods: A 60-year-old female presented with decreased visual acuity on her left eye, ocular hypertension, restricted and painful left abduction, diplopia, swollen eyelids, and orbital discomfort. MRI, as well as ultrasound, showed enlargement in width of medial rectus muscle. After other diagnoses were excluded, the diagnosis of left orbital myositis was established. Results: She was started on systemic corticosteroid treatment, but each time the steroid dose was tapered she experienced a relapse. Immunosuppressive therapy was introduced and the steroid dose was gradually tapered and excluded. One year after immunosuppressive therapy, the clinical findings improved. Conclusion: The diagnosis of orbital myositis requires detailed examination, laboratory testing and MRI scans of the orbits in order to exclude other diseases with similar clinical findings. The first line treatment option is systemic corticosteroid therapy with additional immunosuppressive therapy if needed. Abbreviations: MRI = magnetic resonance imaging, BCVA = best corrected visual acuity, ENT = ear, nose, throat specialist, CBC = complete blood count, WBC = white blood cell, ESR = erythrocyte sedimentation rate, CRP = C reactive protein, HM = hand motion, TED = thyroid eye disease, SLE = systemic lupus erythematosus, ECG = electrocardiogram, CT = computed tomography.

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.

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 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.

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.

Pseudo-Duane retraction syndrome after orbital myositis.

Orbital myositis is a rare, commonly idiopathic, inflammatory condition that affects one or more extraocular muscles. We present a case of unilateral orbital myositis affecting the lateral rectus muscle presenting with gaze-evoked amaurosis, pain, and diplopia, with restrictive limitation of adduction. With improvement in adduction after initiating treatment, we noted narrowing of the palpebral fissure on attempted adduction, mimicking Duane retraction syndrome (DRS). Reported cases of "pseudo-DRS" are associated with multiple etiologies and are characterized by retraction on attempted abduction rather than adduction, as occurs in true DRS. In this case, pseudo-DRS occurred in the setting of idiopathic orbital inflammatory syndrome (orbital myositis) with a motility pattern more consistent with true DRS.

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.

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.

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.

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.