Orbital inflammatory disease (OID) comprises approximately 6% of orbital conditions, affecting individuals across all ages. The range of the primary orbital inflammation\'s differential diagnosis is extensive, encompassing autoimmune disorders such as thyroid diseases, vasculitis, sarcoidosis, connective tissue diseases, immunoglobulin G4-related disease (IgG4-RD), and giant cell myositis, whereas secondary causes span from infections to drug-induced causes. Analyzing histopathological aspects and cell populations could enhance our comprehension of the etiology of orbital inflammatory involvement in systemic diseases such as IgG4-RD. We present a series of four patients from our Rheumatology clinic, each with distinct systemic diseases, illustrating diverse manifestations of OID. This series was conducted to facilitate discussions and diagnoses of challenging cases of OID in a rheumatologic setting. The difficulty in the differential diagnosis arises from the extensive range of structures involved, resulting in a significant variation of clinical manifestations. Furthermore, the lack of definitive diagnostic laboratory tests and, often, histological findings add to the complexity. OID poses diagnostic challenges with variable clinical manifestations and overlapping imaging findings. As a diagnosis of exclusion, a comprehensive evaluation is crucial, often necessitating an orbital biopsy for confirmation. Collaborative efforts among specialists are essential for managing these intricate cases.

UNASSIGNED: Zoledronate is a commonly prescribed medication to maintain bone health; however, a rare side effect includes ocular inflammation. We report a case of simultaneous anterior uveitis and orbital inflammation associated with zoledronate infusion in a patient with metastatic breast cancer. We also performed a literature search to provide an up-to-date summary of cases with zoledronate-associated ocular inflammation.
UNASSIGNED: This is a case report with literature review. Literature search (timeline 2010 to 2023) was performed using PubMed with the search team: (zoledronate) AND (uveitis OR scleritis OR orbital inflammation OR ocular inflammation).
UNASSIGNED: A 48-year-old female presented with left eye pain, swelling, and decreased vision 2 days after receiving zoledronic acid infusion. An ophthalmic exam showed non-granulomatous anterior uveitis. CT orbits and ocular ultrasound showed signs of posterior scleritis and orbital inflammation. Ocular inflammation caused by an infection or metastatic cancer was ruled out. The patient was treated with both topical and systemic corticosteroids. Complete resolution of the inflammation occurred after 2.5 weeks.
UNASSIGNED: Orbital inflammation and uveitis are an uncommon side effect of zoledronate but needs to be promptly recognized and treated to prevent sight-threatening complications.

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.

UNASSIGNED: The current study queries the American Academy of Ophthalmology (AAO) Intelligent Research in Sight (IRIS) registry for data on the epidemiology, work-up, and management patterns of autoimmune orbital inflammation.
UNASSIGNED: Analysis and description of patient data from the IRIS registry between 2013 and 2019 reviewing patients with autoimmune or idiopathic orbital inflammation with filters based on International Classification of Disease (ICD) and Current Procedural Terminology (CPT) codes. Patients with thyroid eye disease, orbital cellulitis, and orbital abscess were excluded.
UNASSIGNED: Demographic descriptions included gender, age, geographic region, and treatment. Sub-analysis was performed by assessing rates of imaging, biopsy, lab work-up, and diagnostic categories.
UNASSIGNED: In a final cohort of 20,584 patients, the mean age of onset of orbital inflammation was 51.7 years; 67% female; and 63% Caucasian, 21% unknown, 12% Black, 2.6% Asian, and 1.5% other. Only 49 had imaging, 78 had laboratory work-up, and 1,411 had biopsy codes. Treatment results showed 166 patients receiving antibiotics, 224 patients receiving steroids, and 35 patients receiving both.
UNASSIGNED: This study assessed the epidemiology, diagnostic patterns, and treatment patterns for orbital inflammation through the AAO IRIS registry. Practise patterns suggest a relatively low overall rate of imaging and laboratory studies compared to biopsies, although this certainly under-represents the actual number of imaging and laboratory studies and exemplifies the inherent imprecision of using a large database. However, the methodology of this study provides a framework of approaching the IRIS registry for oculoplastic research.

UNASSIGNED: Ophthalmic manifestations of varying severity are often associated with systemic autoimmune conditions. Superior orbital fissure syndrome (SOFS) is a rare cranial neuropathy affecting nerves passing through the superior orbital fissure that causes a distinctive pattern of extraocular and pupillary findings. We report the coexistence of SOFS, relapsing polychondritis (RP) and Sjögren\'s syndrome (SS) in a 52-year-old female who presented with a past medical history of hypothyroidism, Raynaud\'s syndrome, and intermittent dry mouth and a 1-week history of worsening chemosis, proptosis, diplopia, and painful ophthalmoplegia.
UNASSIGNED: Following a comprehensive eye examination, the patient underwent a CT head with contrast, MRI of the orbit, lumbar puncture, and laboratory investigations.
UNASSIGNED: CT and MRI examination revealed inflammatory standing in periorbital subcutaneous soft tissues and bilateral exophthalmos with right intraconal fat stranding surrounding the intraorbital and intracanalicular segments of the nerve, respectively. Lumbar puncture and laboratory investigations revealed an elevation in inflammatory biomarkers, a negative infectious workup, and ruled in SS when considering her history alongside a positive Schirmer test. She was started on high-dose steroids, which led to significant improvement; however, treatment revealed type 2 diabetes, necessitating a faster steroid taper, during which there was a reoccurrence of scleritis and ophthalmoplegia, leading to the initiation of rituximab infusions. After completing rituximab course, she was transitioned back to steroid therapy and was successfully tapered without event.
UNASSIGNED: This case is notable for the rare coexistence of SOFS with RP/SS overlap syndrome and highlights the management of concurrent orbital inflammatory syndrome and autoimmune diseases.

UNASSIGNED: Osteopetrosis is a rare heritable disorder characterized by increased bone density resulting from osteoclast dysfunction. Major complications include bone fracture, osteomyelitis, anemia, and cranial nerve compression. Optic atrophy can occur due to compression of the optic nerve. Although osteomyelitis of the jaw is a common complication, it rarely occurs in the maxilla. Here, we report a case of a 74-year-old female with osteopetrosis who developed maxillary osteomyelitis, leading to orbital inflammation.
UNASSIGNED: She was referred to our clinic for 2 months of ptosis and swelling of the left eyelid and temporal region. Previous imaging revealed a left intraorbital occupying lesion, but a biopsy of the temporal subcutaneous tissue did not provide a definitive diagnosis. After 7 months, she presented with severe temporal swelling and purulent discharge. Upon examination, maxillary osteomyelitis resulting from caries of the upper jaw was observed. Treatment with oral antibiotics, drainage of the temporal skin fistula, and regular cleaning of the maxillary drainage improved her symptoms.
UNASSIGNED: This is a rare case of maxillary osteomyelitis associated with osteopetrosis, causing orbital inflammation.

Idiopathic orbital inflammatory disease is a benign, non-infectious, and non-neoplastic space-occupying orbital and peri-orbital inflammation with no identifiable local or systemic causes. Patients usually present with eyelid and periorbital erythema and edema, proptosis, and decreased eye movements. In this report, a case of large serous retinal detachment accompanying a unilateral idiopathic orbital inflammatory disease attack is discussed. It demonstrates that very extensive and highly serous retinal detachment may accompany idiopathic orbital inflammatory disease attacks. Dramatic improvement in clinical findings and imaging can be observed with high-dose steroid treatment in a short time.

We report a rare case of orbital inflammation complicating hemophagocytic lymphohistiocytosis (HLH) patient. HLH is a rare, life-threatening disorder characterized by uncontrolled activation of cytotoxic T lymphocytes, natural killer cells, and macrophages. A 37-year-old man known to have HLH, presented with a left periorbital swelling that was unsuccessfully treated as an orbital cellulitis, with intravenous antibiotics. A computed tomography (CT) scan of the orbits revealed inflammatory changes with no orbital collection or paranasal sinus disease. An orbital biopsy demonstrated lymphoplasmacytic infiltrations admixed with histiocytes. The patient deteriorated and was admitted to the intensive care unit. Ensuing blood results supported a diagnosis of HLH, and the patient responded well to subsequent immunosuppression. This case report highlights the importance of re-considering the diagnosis of orbital cellulitis in treatment resistant cases, particularly in the absence of sinus disease. To our knowledge, this is the third case of orbital inflammation associated with HLH patients.

UNASSIGNED: We report histopathologic orbital tissue analysis from three patients with thyroid eye disease (TED) - active, chronic, and post-teprotumumab to better characterize orbital cellular populations in these varying states of TED.
UNASSIGNED: Orbital tissues in TED demonstrate minimal lymphocytic infiltration in fat and Mueller\'s muscle. Following teprotumumab treatment, the tissues were devoid of lymphocytes with only perivascular cuffs of T-lymphocytes remaining in orbital fat.
UNASSIGNED: In active TED, post-teprotumumab treatment, and in quiescent TED, orbital fat may not show significant inflammatory infiltration. More work is warranted to characterize specific cellular effects of teprotumumab and other biologics.

OBJECTIVE: The purpose of the study was to report three cases of orbital inflammation following administration of the COVID-19 vaccination, manifesting as Tolosa-Hunt syndrome (THS) and orbital myositis.
METHODS: A retrospective case series and literature review of patients who developed orbital inflammation following a COVID-19 vaccination.
RESULTS: One patient presented with Tolosa-Hunt syndrome (THS) 14 days following her third (booster) COVID-19 vaccination, one patient developed orbital myositis 10 days following his first COVID-19 vaccination and one patient developed recurrent orbital myositis 1 and 7 days following her second and fourth COVID-19 vaccination. All patients received the Comirnaty vaccine (Pfizer-BioNTech). A thorough systemic autoimmune disease workup in both patients was unremarkable. Two patients had a prior history of orbital inflammation, with previous involvement of other different orbital structures. Characteristic MRI features for each pathology were observed, supporting the clinical presentation of THS and orbital myositis. There was complete resolution of THS following corticosteroids, with no recurrence at 2 months. Meanwhile, one case of orbital myositis self-resolved at 2 months without use of systemic corticosteroids, while the other patient with orbital myositis required treatment with intra-orbital steroid injections and oral corticosteroids.
CONCLUSIONS: Orbital inflammation has been recognised as a rare adverse effect following COVID-19 vaccination. We present a case series of THS and orbital myositis as varied presentations of this entity.