Review

Authors: Yağmur Seda Yeşiltaş, Ahmet Kaan Gündüz

Idiopathic orbital inflammation (IOI) is a benign inflammatory condition usually confined to the orbit but extraorbital extension can also occur. IOI has been classified into categories including anterior, diffuse, posterior or apical, myositis, and dacryoadenitis. Other rare types of IOI include periscleritis, perineuritis, and focal mass. Diagnosis is based on careful history, clinical findings, computed tomography, and magnetic resonance imaging findings. An orbital biopsy is usually done for accessible orbital lesions such as dacryoadenitis. For other types such as myositis and apical IOI where surgery is difficult or dangerous, orbital biopsy is not initially considered. The mainstay of therapy consists of systemic corticosteroids, but other options including external beam radiotherapy, antimetabolites, alkylating agents, T-cell/calcineurin inhibitors, lymphocyte inhibitors, tumor necrosis factor-α inhibitors, and surgical debulking have also been used.

Topics: Adult; Biopsy; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Orbit; Orbital Pseudotumor; Tomography, X-Ray Computed

PubMed: 30122852
DOI: 10.4103/meajo.MEAJO_44_18

Review

Authors: Luis J Mejico

IgG4-related disease is a fibro-inflammatory condition with tendency to form tumors with inflammatory infiltrate with IgG4 rich plasma cells and elevation of IgG4 level in serum, which may affect virtually every organ and tissue in the organism. IgG4-related ophthalmic disease may present as dacryoadenitis, myositis, other orbital tissues, hypophysitis or pachymeningitis causing cranial neuropathies. The diagnosis of IgG4-related disease is based on a typical clinical scenario, supportive laboratory data, expected radiological characteristics and distinct histopathological and immunohistochemical features. Corticosteroid followed by the use of long-term immunosuppressive therapy is the most commonly attempted treatment.

PubMed: 25859140
DOI: 10.1016/j.sjopt.2014.09.008

Review

Authors: Alvita J Chan, Amandeep S Rai, Shirley Lake...

Orbital myositis is a rare manifestation of systemic lupus erythematosus (SLE). Herein, we report a case of orbital myositis in a patient with SLE, along with a literature review. A 45-year-old female patient presented with pain in the right eye, chemosis, proptosis, and limited abduction. Computed tomography of her orbits revealed thickening of her right lateral rectus muscle. She had no other systemic symptoms. There was no elevation in the biomarkers of inflammation or disease activity. She was treated with high-dose steroids, and her symptoms resolved rapidly. It is important to maintain a high index of suspicion for orbital myositis in patients with SLE even when there are no systemic disease activities, such that early treatment can be initiated. It is also important to rule out other mimickers such as orbital cellulitis and thyroid eye disease.

PubMed: 32809933
DOI: 10.5152/eurjrheum.2020.19217

Authors: Aida Pidro, Admira Dizdarević, Nina Jovanović...

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. 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. 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. 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. 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.

Topics: Adrenal Cortex Hormones; Diplopia; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Oculomotor Muscles; Orbital Myositis; Steroids

PubMed: 35935078
DOI: 10.22336/rjo.2022.34

Review

Authors: Piraye Oflazer

Giant cell myositis (GCMm) and giant cell myocarditis (GCMc) are two rare autoimmune conditions. Among these, GCMc is a life-threatening disease with a 1-year mortality rate of 70%. Lethal ventricular arrhythmias, rapid evolution to heart failure and sudden death risk makes GCMc an emergency condition. It is thought to be mediated by T-cells and characterized by the presence of myofiber necrosis and giant cells in biopsies. Most commonly co-manifesting conditions with GCMm and/or GCMc are thymoma, myasthenia gravis and orbital myositis, all of which are treatable. As suspicion is the key approach in diagnosis, the physician following patients with thymoma with or without myasthenia gravis and with orbital myositis should always be alert. The fatal nature of GCMc associated with these relatively benign diseases deserves a special emergency attention with prompt institution of combined immunosuppressive treatment and very early inclusion of heart failure teams.

Topics: Giant Cells; Humans; Myocarditis; Myositis

PubMed: 33458585
DOI: 10.36185/2532-1900-033

Authors: Terence Ang, Viraj Chaggar, Jessica Y Tong...

We performed a comprehensive systematic review to identify medication-associated orbital inflammation and to characterize its clinico-radiological features. We reviewed English-language articles describing medication-associated orbital inflammation (i.e., orbital myositis, dacryoadenitis and orbital fat) published to June, 2023. Isolated inflammation of the intraocular structures or globe alone (i.e. uveitis, scleritis, optic neuritis and perineuritis) were excluded. In medication-associated orbital inflammation, the extraocular muscles are preferentially affected, occurring in isolation or in combination with other orbital and/or intraocular structures. Clinico-radiological manifestations may be non-specific; however, certain medications may be distinguished according to the presence of systemic prodrome, laterality, associated intraocular inflammation, and predisposition to involve certain orbital structures. Rapid identification, discontinuation of the provoking medication, and systemic corticosteroid therapy (if appropriate) typically achieves a favorable visual prognosis. As new medications become adopted by clinicians, rare adverse effects will be further delineated.Medication-associated orbital inflammation is an important diagnostic consideration in orbital inflammatory disease. A careful medication history and clinical assessment may be revealing, permitting timely discontinuation of the offending agent and initiation of appropriate management.

Topics: Humans; Dacryocystitis; Glucocorticoids; Orbital Diseases; Orbital Myositis

PubMed: 38490453
DOI: 10.1016/j.survophthal.2024.03.003

Review

Authors: J Gonzalez Barlatay, C Pagano Boza, G V Hernandez Gauna...

The aim of this review was to describe orbital inflammation secondary to aminobisphosphonates by analyzing demographic data, clinical presentation, and treatment of the disease. This is a narrative literature review. The search was performed using databases such as Ovid/MEDLINE and COCHRANE. The searches were limited to papers in the English language. We found 43 cases of orbital inflammation due to aminobisphosphonates. Zoledronate was the drug most associated with orbital side effects. Clinical presentation was evident by unilateral involvement (89%), palpebral edema (88%), conjunctival congestion (81%), chemosis (79%), ocular pain (77%), ocular motility impairment (65%), proptosis (56%), and blurred vision (39%). It can affect both eyes (11%) and is accompanied by anterior uveitis (23%). Orbital inflammation secondary to aminobisphosphonates is a severe side effect. Clinically, it cannot be distinguished from idiopathic inflammation of the orbit. Therefore, it is important to rule out previous drug exposure. Timely treatment is vital to expect a favorable outcome, with systemic corticosteroids being the treatment of choice.

PubMed: 35371416
DOI: 10.18502/jovr.v17i1.10176

Review

Authors: T Aguiar, A Morganho, V Passão...

Orbital myositis is a subgroup of the nonspecific inflammatory syndrome or orbital pseudotumor and is characterized by a primary inflammation of extraocular muscles. The authors describe a 70-year-old patient with acute proptosis, ocular pain and right ophthalmoplegia, whose orbital computed tomographic scan showed enlargement of the homolateral extraocular muscles. Clinical presentation and complementary tests were compatible with the diagnosis of orbital myositis however, because of the particular aspects, which included retinal central vein occlusion, optic nerve lesion, distension of the superior ophthalmic vein and the homolateral cavernous sinus, the differential diagnosis with cavernous sinus pathology and thyroid ophthalmopathy was considered. The importance of a rapid diagnosis and treatment is stressed.

Topics: Aged; Female; Glucocorticoids; Humans; Neurologic Examination; Orbit; Orbital Pseudotumor; Prednisolone; Tomography, X-Ray Computed

PubMed: 9446484
DOI: No ID Found

Authors: Antonio Vitale, Valeria Caggiano, Eduardo Martin-Nares...

VEXAS syndrome is a recently described monogenic autoinflammatory disease capable of manifesting itself with a wide array of organs and tissues involvement. Orbital/ocular inflammatory manifestations are frequently described in VEXAS patients. The objective of this study is to further describe orbital/ocular conditions in VEXAS syndrome while investigating potential associations with other disease manifestations. In the present study, twenty-seven out of 59 (45.8 %) VEXAS patients showed an inflammatory orbital/ocular involvement during their clinical history. The most frequent orbital/ocular affections were represented by periorbital edema in 8 (13.6 %) cases, episcleritis in 5 (8.5 %) patients, scleritis in 5 (8.5 %) cases, uveitis in 4 (6.8 %) cases, conjunctivitis in 4 (6.8 %) cases, blepharitis in 3 (5.1 %) cases, orbital myositis in 2 (3.4 %) cases. A diagnosis of systemic immune-mediated disease was observed in 15 (55.6 %) cases, with relapsing polychondritis diagnosed in 12 patients. A significant association was observed between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome (Relative Risk: 2.37, 95 % C.I. 1.03-5.46, p = 0.048). Six deaths were observed in the whole cohort of patients after a median disease duration of 1.2 (IQR=5.35) years, 5 (83.3 %) of which showed orbital/ocular inflammatory involvement. In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed. This makes ophthalmologists a key figure in the diagnostic process of VEXAS syndrome. The high frequency of deaths observed in this study seems to suggest that patients with orbital/ocular involvement may require increased attention and more careful follow-up.

Topics: Humans; Male; Female; Adult; Registries; Middle Aged; Young Adult; Adolescent; Orbital Diseases; Hereditary Autoinflammatory Diseases; Eye Diseases; Child; Aged; Scleritis; Polychondritis, Relapsing

PubMed: 38554594
DOI: 10.1016/j.semarthrit.2024.152430