IgG4-related disease in patients with idiopathic orbital inflammation

Background To identify the prevalence of positive IgG4 immunostaining in orbital tissue among patients previously diagnosed with nongranulomatous idiopathic orbital inflammation (IOI) and to compare the clinical characteristics of patients with and without IgG4-positive cells. Methods A retrospective review of all patients with a histopathologic diagnosis of IOI was performed. Immunohistochemical staining was performed to identify IgG-positive cells and IgG4-positive cells. Multivariate analysis was performed using likelihood ratio-test logistic regression on the differences between IgG4-related disease (IgG4-RD) and non-IgG4-RD. Results Of the 45 patients included, 21 patients (46.7%) had IgG4-positive cells, with 52.4% being male and a mean age of 55.9 ± 13.4 years. Bilateral ocular adnexal involvement (adjusted odds ratio [aOR] = 9.45; P = 0.016) and infraorbital nerve enlargement (aOR = 12.11; P = 0.008) were frequently found in IgG4-RD patients. Complete remission occurred in 23.8% of IgG4-RD patients and 41.7% of non-IgG4-RD patients. IgG4-RD patients had more frequent recurrent disease than non-IgG4-RD patients. Conclusions Nearly 50% of IgG4-RD patients were previously diagnosed with biopsy-proven IOI. IgG4-RD was more frequent in patients with bilateral disease and infraorbital nerve enlargement, showing the importance of tissue biopsy in these patients. Immunohistochemistry studies of all histopathology slides showing nongranulomatous IOI are highly recommended to evaluate for IgG4-RD.

A Case of Idiopathic Orbital Inflammation Presenting as Isolated Myositis of the Inferior Oblique Muscle

Purpose: To report a case of idiopathic orbital inflammation presenting with isolated myositis of the inferior oblique muscle. Case summary: A 54‐year‐old man presented with swelling on the left lower lid, pain on superonasal and inferonasal gaze, and binocular diplopia for 2 months. His head was tilted to the right by about 5° and mild conjunctival injection and 3 mm narrowing of palpebral fissure of the left eye compared to the other eye were observed. Eight prism diopter (PD) left hypertropia and 4 PD intermittent esotropia were noted on primary gaze, which worsened on leftward gaze, downward gaze, and left head tilt. Orbital magnetic resonance imaging (MRI) showed enhanced hypertrophy of the left inferior oblique muscle. Systemic work‐up for possible inflammatory diseases yielded negative results. Therefore, a presumptive diagnosis of idiopathic isolated myositis of the left inferior oblique muscle was made. The patient was treated with 60 mg of oral corticosteroid per day for the first week, and the dose was tapered for 1 month as the symptoms reduced. Two months later, the patient became free from any symptoms and follow-up orbital MRI showed a significant decrease in size of the left inferior oblique muscle. There have been no signs of recurrence for 7 months. Conclusions: A presumptive diagnosis of idiopathic isolated myositis of the inferior oblique muscle was made in a patient with swelling of the left lower lid and binocular diplopia based on orbital MRI and systemic work‐up. Good results were achieved with oral corticosteroid therapy.

Increased Dysfunctional and Plastic Regulatory T Cells in Idiopathic Orbital Inflammation

BackgroundIdiopathic orbital inflammation (IOI) is a disfiguring and vision-threatening fibroinflammatory disorder. The pathogenesis of IOI has not been elucidated. We sought to clarify the regulatory T cell (Treg) distribution and function in patients with IOI.MethodsThe frequency, phenotype and function of Tregs were identified by multicolor flow cytometry and in vitro cell functional assays. Plasma and tissue samples were obtained to investigate cytokines, chemokines and their receptors of interest by relative real-time polymerase chain reaction (PCR) and Luminex assays.ResultsCompared with healthy subjects, patients with IOI exhibited obvious increases of Tregs in peripheral blood and affected orbital tissues. Circulating Tregs from patients with IOI were significantly more polarized to a Th17-like phenotype with defective regulatory function, whereas orbit-derived Tregs were polarized to a Th2-like phenotype. Furthermore, ST2 expression levels in circulating Tregs and interleukin (IL)-33 mRNA levels in orbital tissues were decreased in IOI. IL-33 restored the suppressive function of Tregs, reduced interferon (IFN)-γ production by Tregs and decreased the activation of orbital fibroblasts (OFs) cocultured with Tregs in IOI.ConclusionIncreased Tregs with proinflammatory and profibrotic polarization were first identified in IOI, suggesting that Treg plasticity and heterogeneity plays an essential role in IOI pathogenesis. Additionally, our study identified a regulatory effect of IL-33 on inflammation and fibrosis in IOI. Reversing the plastic Tregs via IL-33 might be a potential option for IOI patients.

Orbital aspergillosis: a case report and review of the literature

Background Orbital aspergillosis is a rare sight- and life-threatening fungal infection affecting immunocompromised or otherwise healthy patients. It is often misdiagnosed due to its unspecific clinical and radiologic appearance. Therapeutic delay can have dramatic consequences. However, progress in microbiological diagnostic techniques and therapeutic experience from case series help improve the management of this disease. Case presentation A 78-year-old immunocompetent woman presented at an eye clinic for subacute swelling, reddening, and ptosis of her left upper eyelid. Based on radiologic and histologic considerations, she was treated for idiopathic orbital inflammation, but her condition worsened. After a second biopsy of the orbital mass, aspergillosis was diagnosed. Her condition improved promptly after initiation of an oral voriconazole treatment. Additionally, using a polymerase chain reaction (PCR) assay, A. fumigatus was identified on tissue of both biopsies and its azole susceptibility was examined simultaneously. Conclusions In the case described here, oral antifungal treatment was sufficient for the therapy of invasive orbital aspergillosis. Performing fungal PCR on orbital tissue can accelerate the diagnostic process and should be performed in ambiguous cases of slowly growing orbital mass. Finally, interdisciplinary management is the key to optimal treatment of orbital tumours and infections.