Idiopathic Sclerosing Orbital Inflammation Research Articles

CASE REPORT: IDIOPATHIC SCLEROSING ORBITAL INFLAMMATION

Idiopathic sclerosing orbital inflammation (ISOI) also known as sclerosing orbital pseudotumor is a rare, idiopathic, chronic, slowly progressive orbital inflammation, characterized by scarring of the tissue that represents a mass effect. The latter leads to bulging of the eye, restriction of ocular movements and visual impairment up to potential loss of vision. It is a subtype of non-specific inflammatory orbital pseudotumor, which both radiologically and clinically mimics a malignant process or other orbital inflammatory disease, and therefore is challenging to diagnose and manage. This case highlights the challenges in diagnosis and management of ISOI.

Acute Orbital Apex Syndrome Caused by Idiopathic Sclerosing Orbital Inflammation.

Idiopathic sclerosing orbital inflammation (ISOI) is a distinct entity among other orbital diseases. It is characterized by marked fibrosis and inflammatory cell infiltration that can damage orbital structures. Clinical manifestations were variable, including ocular and periocular redness, proptosis, and pain. Ocular motor restrictions and optic nerve dysfunction might occur in severe cases. We herein report a patient of ISOI who presented with total ophthalmoplegia and acute vision loss. His symptoms were relieved mainly as his vision improved to 20/25 after receiving corticosteroid and immunosuppressant therapies. Therefore, ISOI should be one of the deferential diagnoses when we encounter cases with acute orbital apex syndrome. With prompt evaluation and in-time treatment, a favorable outcome is possible.

Re: "Clinical Outcomes of IgG4-Related Ophthalmic Disease and Idiopathic Sclerosing Orbital Inflammation".

The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Bulent Yazici, M.D., Department of Ophthalmology, Uludag University, Bursa 16059, Turkey. E-mail: [email protected].

Reply Re: "Clinical Outcomes of IgG4-Related Ophthalmic Disease and Idiopathic Sclerosing Orbital Inflammation".

Reply Re: "Clinical Outcomes of IgG4-Related Ophthalmic Disease and Idiopathic Sclerosing Orbital Inflammation".

Clinical Outcomes of IgG4-Related Ophthalmic Disease and Idiopathic Sclerosing Orbital Inflammation.

To compare the clinical features, treatment outcomes, and prognoses of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and idiopathic sclerosing orbital inflammation (ISOI). This retrospective case series included 40 and 22 biopsy-proven cases of IgG4-ROD and ISOI, respectively. The authors reviewed medical charts and images to determine the clinical presentation, involvement pattern, and treatment outcomes. The main outcome measures were differences in disease manifestation, treatment modalities and efficacy, medication-free remission rate, and predictive factors for remission. The median ages were 51 (range 31-72) and 39 (range 9-73) years in the IgG4-ROD and ISOI groups, respectively. The most frequently involved sites were the lacrimal gland (39 cases, 98%) in IgG4-ROD patients and the extraconal region (15 cases, 68%) in ISOI patients. No significant difference was observed in the initial treatment response between the groups. Medication-free remission was achieved in 22 (55%) IgG4-ROD patients and 10 (45%) ISOI patients; none of the IgG4-ROD patients showed permanent ocular dysfunction, while half of the ISOI patients had permanent visual loss or limited ocular motilities. Multivariate analysis indicated that extraocular muscle enlargement (odds ratio, 0.11; 95% confidence interval, 0.01-0.98) and IgG4:IgG ratio on histopathology (odds ratio, 0.08; 95% confidence interval, 0.01-0.86) were negatively associated with medication-free remission in IgG4-ROD patients. Although IgG4-ROD and ISOI share common histological findings of mass-forming sclerosis, IgG4-ROD frequently has a more indolent clinical course, whereas ISOI often demonstrates a more aggressive clinical course, resulting in ocular dysfunction even after remission. Different pathogeneses may account for the different disease characteristics.

Rituximab Therapy for Recalcitrant Idiopathic Sclerosing Orbital Inflammation.

Three patients (3 female patients; aged 7, 35, and 61 years) who had recalcitrant idiopathic sclerosing orbital inflammation were treated with rituximab. The disease was bilateral in 1 patient (4 orbits in total): diffuse in 2 and localized in 2 orbits. It caused optic neuropathy in 1 orbit of each patient. Conventional immunotherapy and tumor debulking surgery were unsuccessful in controlling the disease. After rituximab infusions (375 mg/m2/week for 4 weeks), all patients improved symptomatically. Radiologically, the local lesions resolved completely and diffuse lesions partially. Two patients with recurrent inflammation during follow up (78, 58, and 51 months) responded well to immediate, short-term steroid treatments. Short-term rituximab therapy can induce effective remissions in patients with refractory idiopathic sclerosing orbital inflammation. Early and local lesions may respond better to treatment than diffuse lesions. Nevertheless, inflammatory exacerbations can occur during late follow up.

IgG4-related Ophthalmic Disease in Idiopathic Sclerosing and Non-Sclerosing Orbital Inflammation: A 25-Year Experience

ABSTRACTPurpose: To determine the prevalence, clinical manifestations, and treatment outcomes of IgG4-related ophthalmic disease (IgG4-ROD) in previously diagnosed idiopathic orbital inflammation (IOI).Materials and Methods: Thirty one surgically treated patients with IOI between 1990 and 2015 were divided into sclerosing (SIOI) and non-sclerosing (NSIOI) to identify IgG4-ROD. Preserved pathological specimens were immunostained for IgG4 cells. Changes in clinical features were evaluated before and after treatment.Results: Out of the 31 patients, 15 (48.4%) had IgG4-ROD, consisting of 7 of the 16 SIOI (43.8%) and 8 of the 15 NSIOI patients (53.3%). Among 15 patients with IgG4-ROD, 4 (26.7%) achieved complete remission, which was significantly less than among IgG4-unrelated patients (11/16, 68.8%, p = .03). Furthermore, 3 out of 15 IgG4-ROD patients (20%) experienced recurrence, all of whom had SIOI, compared to 0% among IgG4-unrelated patients (p = .1).Conclusions: IgG4-ROD is common among previously identified IOI in our study. Treatment response is modest in IgG4-ROD patients.

IgG4-related disease in idiopathic sclerosing orbital inflammation

AimsTo investigate the frequency of IgG4-related disease (IgG4-RD) among patients previously diagnosed with idiopathic sclerosing orbital inflammation (ISOI), and to compare the clinical features and treatment outcomes of patients with...

Idiopathic Sclerosing Orbital Inflammation

We read with great interest the report by Levin et al (1) regarding the role of the optic nerve biopsy in the management of progressive optic neuropathy. We had the opportunity to evaluate a 50-year-old man with progressive deterioration of vision in his left eye for 2 months with minimal periocular pain. Visual acuity was 20/20, right eye, and no light perception, left eye. The right fundus was normal but there was marked optic disc edema in the left eye (Fig. 1). Magnetic resonance imaging (MRI) of the brain and orbits demonstrated enhancement and thickening of the left optic nerve (Fig. 2). An extensive work-up including hematologic tests, lumbar puncture, and computed tomography of chest, abdomen, and pelvis was unremarkable. The patient was given 1 g of methylprednisolone intravenously for 3 days followed by a tapering dose of oral steroids over several months. Although there was improvement in the left optic disc edema, vision remained unchanged.FIG. 1: Left fundus shows marked optic disc edema with peripapillary hemorrhages.FIG. 2: Postcontrast axial (A) and coronal (B) T1 magnetic resonance imaging with fat suppression shows thickening and enhancement of the left optic nerve.Four months after onset of symptoms, the patient reported vision loss in the right eye. Acuity was 20/40, right eye, and no light perception, left eye. Funduscopy revealed right optic disc edema and left optic atrophy. MRI demonstrated thickening of the right optic with marked enhancement following intravenous contrast. Prednisone was restarted at a dose of 1 mg/kg/d, and there was rapid improvement in the vision in the right eye with resolution of right optic disc edema. Given involvement of the previously unaffected right eye, a biopsy of the left optic nerve was performed. Histopathology showed primarily fibrosis and an inflammatory infiltrate consisting of lymphocytes, histiocytes, and plasma cells (Fig. 3). No granulomas were identified. Stains for infectious, immune, and neoplastic disorders were nondiagnostic. Based on these results, a diagnosis of idiopathic sclerosing orbital inflammation (ISOI) was made.FIG. 3: Biopsy demonstrates optic nerve and sheath fragments with marked fibrosis accompanied by an inflammatory infiltrate of lymphocytes, histiocytes, and plasma cells (periodic acid–Schiff, ×400).ISOI is a rare condition characterized by marked fibrosis and some inflammatory infiltrate (2–4). However, unlike idiopathic orbital inflammation, which has an acute clinical onset, the progression of ISOI generally has a chronic and indolent course (5). In our patient, there was consecutive involvement of both optic nerves. There are reports of ISOI associated with systemic idiopathic fibrosis and increased serum levels of IgG4 (6,7). Our patient did not show increased serum levels of IgG4, and the biopsy specimen was negative for the presence of IgG4.

AB1233 Experience in treatment of a cohort of chinese patients with immunoglobulin G4-related systemic disease

BackgroundImmunoglobulin G4-related systemic disease (IgG4-RSD) is a rapidlyemerging disease characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis(1). As...

I feel it in my bones: a rare presentation of idiopathic sclerosing orbital inflammation with hyperostosis

I feel it in my bones: a rare presentation of idiopathic sclerosing orbital inflammation with hyperostosis

Inflamação esclerosante idiopática da órbita: estudo clinico-patológico

OBJETIVO: Descrever oito casos de inflamacao esclerosante idiopatica da orbita, incluindo a analise clinica e histopatologica. METODOS: Estudo retrospectivo atraves do livro de registros do Laboratorio de Patologia Ocular (LPO) do Hospital Universitario Professor Edgard Santos, Universidade Federal da Bahia, no periodo de 1974 a 2010. Alem de revisao de lâminas de todos os casos e das imagens clinicas e de exames complementares recuperadas dos arquivos pessoais dos autores. RESULTADOS: Dentre o total de especimes submetidos ao LPO (7546) apenas 8 casos receberam diagnostico final de inflamacao esclerosante idiopatica da orbita. Em todos os casos houve piora ou deterioracao da acuidade visual a despeito do tratamento, com excecao de um, que permaneceu com a mesma visao (percepcao luminosa). Em nosso estudo, observamos envolvimento orbitario predominantemente apical. Todos os nossos pacientes foram submetidos a biopsia incisional com finalidade diagnostica. Tambem submeteram-se a corticoterapia e em um caso foi associada a radioterapia anti-inflamatoria; em dois destes casos, foi utilizado tambem ciclofosfamida. Dois pacientes com acuidade final sem percepcao luminosa tiveram que ser submetidos a exenteracao parcial da orbita para tratamento de dor incontrolavel. CONCLUSAO: A inflamacao esclerosante orbitaria idiopatica permanece como uma doenca pouco conhecida, de pessimo prognostico visual.

Radiotherapy With or Without Surgery for Patients With Idiopathic Sclerosing Orbital Inflammation Refractory or Intolerant to Steroid Therapy

To evaluate the outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) treated with radiotherapy with or without surgery. We retrospectively reviewed 22 patients with histopathologically confirmed ISOI who had been refractory or intolerant to steroid therapy and treated with radiation with or without surgery. The radiation dose ranged from 20 to 40 Gy (median, 20 Gy) at 2Gy per fraction. Presenting signs and treatment outcomes were assessed. Proptosis was the most common sign at presentation, seen in 19 (86.3%) patients, followed by restriction of extraocular movements in 10 (45.4%) patients. Response to radiotherapy was complete in 15 (68.1%) patients, partial in 3 (13.6%) patients, and none in 4 (18.2%) patients. At the median follow-up of 34 months, 14 (63.6%) patients had progression-free state of symptoms and signs, with the progression-free duration ranging from 3 to 75 months (median, 41.5 months), whereas 8 (36.4%) patients had recurrent or persistent disease although they hadreceived radiotherapy. Of the 14 progression-free patients, 6 underwent a bimodality treatment of debulking surgery of ocular disease and radiotherapy. They had had no recurrent disease. Cataract was the most common late complications, and 2 patients experienced a Grade 3 cataract. Our study suggests that for patients with ISOI who are refractory or intolerant to steroid therapy, 20 Gy of radiotherapy appears to be effective for the control of disease with acceptable complications, especially when it is combined with surgery.

Idiopathic Sclerosing Orbital Inflammation

To characterize clinical features, diagnostics studies, treatments, and outcomes of patients with histologically proven idiopathic sclerosing orbital inflammation (ISOI), to define optimal management for this recalcitrant disease, and to determine changes in characterization and management by comparing our results with the last significant literature review. A search of the U.S. National Library of Medicine: National Institutes of Health's electronic database for cases and case series in the English literature of biopsy-proven ISOI published between March 1994 and September 2010 was conducted. A cross-literature review was performed to tabulate demographics, clinical findings, studies, treatments, and outcomes, which were compared with the ISOI data published by Rootman et al. (1994). Sixty-one cases, 71 eyes from 17 published reports, met inclusion criteria. No ethnic, sex, or comorbidity predilection was established. Patients typically presented in the fourth decade with proptosis (73%), pain (49%), and normal vision (44%). Orbital imaging and histopathology were sparsely reported. Most common treatments involved systemic corticosteroids either alone (34%) or combined with other modalities (51%). Characteristics of the disease remain unchanged, and best management was not determined due to inconsistent reporting methods across the literature. Collaboration with established groups (i.e., European Group On Graves Orbitopathy (EUGOGO), International Thyroid Eye Disease Society (ITEDS)) or the formation of a new group of physicians and scientists to help develop a systematic approach for future reporting and evaluation was proposed.

Idiopathic Sclerosing Inflammation Presenting as Sinusitis

Idiopathic sclerosing orbital inflammation is a rare finding that is poorly delineated, immune mediated, and causes severe symptoms and disability. It has been described affecting the orbit in addition to other sites within the head and neck, but has rarely been described presenting as sinusitis. A case report and literature review were performed. A 14-year-old girl with right-sided face and eye pain and pressure for >1 month presented 3 days after endoscopic sinus surgery for presumed acute sinusitis. She subsequently developed ipsilateral vision loss and hypesthesia of the infraorbital nerve. MRI revealed a mildly enhancing soft tissue intensity lesion extending from the right maxillary sinus into the pterygopalatine fossa and orbital apex through the inferior orbital fissure. Biopsy specimens of the lesion were consistent with a sclerosing inflammatory lesion. High-dose steroids led to rapid improvement in vision and pain; however, the patient was unable to tolerate steroid weaning because of recurrence of eye pain and headache. Repeat imaging showed progression of the lesion. Rheumatology was consulted and the patient's steroid therapy was altered and her medications were expanded to include azathioprine. The patient's symptoms improved and subsequent imaging showed a reduction in the size and extent of the lesion. Idiopathic sclerosing inflammation is characterized by primary, chronic, and immunologically mediated fibrosis. Patients typically have a poor response to corticosteroid treatment or radiotherapy. Immunosuppressive therapy in addition to corticosteroids is the recommended treatment.

Suspected idiopathic sclerosing orbital inflammation presenting as immunoglobulin G4-related disease: a case report

IntroductionIdiopathic sclerosing orbital inflammation is a rare and ill-defined heterogeneous entity, and a distinct subset of orbital inflammation. Recently, attention has been focused on immunoglobulin G4-related disease complicated with fibrotic changes in some other organs with high serum immunoglobulin G4 levels. This report presents a case of suspected idiopathic sclerosing orbital inflammation complicated with high serum immunoglobulin G4 levels.Case presentationAn 82-year-old Japanese woman had a 30-year history of chronic thyroiditis. She experienced right ptosis and eyelid swelling. These symptoms gradually developed over five years. The clinical and radiographic findings suggested that our patient had idiopathic sclerosing orbital inflammation. We were unable to obtain our patient's consent to perform a biopsy. While the serum immunoglobulin G level was within the normal limits, the serum immunoglobulin G4 level was significantly elevated. The serum immunoglobulin G4 levels decreased after the administration of oral prednisolone at a daily dose of 20 mg. In addition, the swelling and ptosis of the right upper eyelid disappeared gradually after four weeks. Our patient was then suspected to have idiopathic sclerosing orbital inflammation complicated with immunoglobulin G4-related disease and chronic thyroiditis.ConclusionAn orbital pseudotumor of this type is indicative of idiopathic sclerosing orbital inflammation immunoglobulin G4-related disease. Immunoglobulin G4 may thus be considered a subclass of immunoglobulin G when the serum immunoglobulin G level is within normal limits.

Idiopathic Sclerosing Orbital Inflammation with Intranasal Extension

Introduction: To report a case of idiopathic orbital sclerosing inflammation (ISOI) with intranasal extension.Material and Methods: The patient presented with a 6-month history of epiphora, upper eyelid swelling, ptosis and mild orbital pain. Ophthalmologic examination, CT, MRI and biopsy with surgical debulking were performed.Results: MRI revealed a homogeneously enhancing diffuse right orbital mass in the inferonasal quadrant of the orbit, which extended to the nasal cavity up to inferior nasal concha, maxillary and ethmoid sinuses. Histological analysis showed dense collagenous tissue with sparse infiltration of mixed inflammatory cells. Inmunohistochemical analysis confirmed polyclonality. The diagnosis of idiopathic sclerosing orbital inflammation was made and 80 mg/day of oral prednisolone was prescribed. At last follow up, one year later, there was no clinical evidence of recurrent orbital disease.Conclusion: ISOI can present with extraorbital extension. Corticosteroids are a reasonable first-line treatment, until the pathogenesis is better understood.

Treatment of idiopathic sclerosing inflammation of the orbit (myositis) with infliximab

We report the successful use of infliximab in a young patient with idiopathic sclerosing orbital inflammation (myositis). We describe the patient's medical records and review the literature. Idiopathic myositis in this patient could not be satisfactorily treated with corticosteroids or chemotherapy. Biopsy showed sclerosing orbital inflammation. The patient had a prompt and sustained response with infliximab and methotrexate treatment. Infliximab and methotrexate might be considered in patients with idiopathic sclerosing orbital inflammation (myositis).

Idiopathic Sclerosing Orbital Inflammation: Two Cases Presenting With Paresthesia

The authors report 2 patients with idiopathic sclerosing inflammation of the orbit who presented with periorbital paresthesia in the trigeminal nerve distribution. The diagnosis in both cases was confirmed with biopsy and both patients responded to corticosteroid treatment. Although periorbital paresthesia is usually a sign of malignancy, these cases illustrate that it may also occur in patients with sclerosing orbital inflammation.

Idiopathic Sclerosing Orbital Inflammation

To perform a multicenter review of the clinical features and treatment of 31 patients with idiopathic sclerosing orbital inflammation. We included all patients with histologically confirmed idiopathic sclerosing orbital inflammation from 5 regional orbital centers. We reviewed the case notes to determine the clinical presentation, diagnostic features, and response to treatment. The main outcome measures were duration and nature of symptoms, anatomical location of disease, histopathological findings, treatment modalities, treatment efficacy and adverse effects, and final clinical status. We included 13 male and 18 female patients ranging in age from 7 to 83 years. The average duration of symptoms at presentation was 13.4 months. There was a predilection for the lateral and superior quadrants. Thirteen patients had apical disease, and 4 had extraorbital involvement. Histopathological findings invariably showed sclerosis associated with a sparse mixed cellular infiltrate. Twenty-seven patients were treated with oral prednisolone, response to which was good in 9 patients, partial in 11, and poor in 7. Six patients were treated with a second-line immunosuppressive agent, and 6 received radiotherapy. The response to radiotherapy was generally poor. Idiopathic sclerosing orbital inflammation is a rare condition that can be difficult to diagnose and manage. Early intervention with immunosuppression in the form of corticosteroids combined with second-line agents can result in control and even regression of the disease.