Histopathologic Findings in Idiopathic Orbital Myositis.

PURPOSE: To report the histologic and clinical features of idiopathic orbital myositis (IOM) patients who underwent extraocular muscle (EOM) biopsy over 20 years, to provide the first methodical reference to the expected histopathologic findings, and to discuss the histopathologic differences from common differential diagnoses. DESIGN: Cohort study. PARTICIPANTS: All patients with a diagnosis of IOM who underwent EOM biopsy from 2000 through 2019 were included. Patients who had a different final diagnosis were excluded. METHODS: Tissue samples of EOM and medical records of all participants were reviewed. MAIN OUTCOME MEASURES: Histopathologic features of muscle biopsy, including tissue morphologic features and cellular composition. RESULTS: Thirteen patients met both inclusion and exclusion criteria, and their tissue samples were revisited. Nine patients showed histopathologic findings that suggested a conclusive diagnosis of IOM, and the study focused on them. The average age at presentation was 49 years, and 66.7% of patients were women. The most commonly biopsied EOM was the medial rectus (44.4%). The most common indications for biopsy were nonresolving orbital disease with inadequate response to corticosteroids (44.4%) or a high suspicion of malignancy because of known pre-existing systemic malignancy or the presence of an atypical orbital mass in addition to enlarged muscles (44.4%). The histopathologic findings that suggested a diagnosis of IOM were splaying of muscle fibers by inflammatory infiltrates (n = 9) and mild fibrosis (n = 8) in the endomysium or replacing muscle fibers, with no granulomas or vasculitis. The inflammatory infiltrates identified were of chronic inflammatory cells, consisting of lymphocytes (n = 9), plasma cells (n = 6), and histiocytes (n = 6). Other less commonly identified cells were eosinophils (n = 4), polymorphonuclears (n = 1), and giant cells (n = 1). Muscle fiber degeneration or regeneration was evident in 5 patients. Four patients from the initial cohort showed inconclusive histologic findings on revision and were reassigned as suspected IOM. CONCLUSIONS: The histopathologic features of involved muscles in IOM resemble those seen in idiopathic orbital inflammation and differ from those seen in common differential diagnoses. Extraocular muscle biopsy should be strongly considered whenever the presentation of orbital myositis is not typical or when significant underlying conditions are a possibility.

Case Report: Orbital Myositis and Myasthenia Gravis as Symptoms of Immune Reconstitution Inflammatory Syndrome in a Patient With Human Immunodeficiency Virus Infection.

We present a case of a 37-year-old man with HIV infection who had been on antiretroviral therapy for one year. He was admitted to our hospital with red and swollen eyes, acute onset progressive exophthalmos, and intermittent diplopia endured for 7 days. His symptoms, exam, and imaging led to a diagnosis of immune reconstitution inflammatory syndrome associated orbital myositis. His symptoms improved considerably after glucocorticoid therapy. Following a reduction in the oral prednisone dose, he re-presented with left ptosis, which rapidly progressed to bilateral ptosis. Diagnostic testing led to the diagnosis of immune mediated myasthenia gravis. Treatment with pyridostigmine bromide, prednisone, and tacrolimus was initiated. One month later, the patient's symptoms improved significantly. There was a probable association between his symptoms and autoimmune immune reconstitution inflammatory syndrome. This report highlights the importance of recognizing autoimmune disorders in human immunodeficiency virus-infected patients undergoing antiretroviral therapy. Orbital myositis and myasthenia gravis in human immunodeficiency virus-infected patients correlate closely with immunity status following a marked increase in CD4+ T cell counts.

Idiopathic inflammatory diseases of orbit and ocular adnexa: Histopathological and immunochemical analysis.

Purpose: : To present histopathological and immunohistochemical analysis of idiopathic inflammatory diseases of orbit and ocular adnexa. Methods: Design- A retrospective laboratory-based study. The study was carried out in an ocular pathology laboratory in a tertiary institute of northeast India where analysis of 93 cases was done in 5 years, during the period from 2011 to 2016. Hematoxylin--eosin and special stains were done for the diagnoses. Immunohistochemistry (IHC) panel was also carried out. For infectious pathology, Grocott's methenamine silver (GMS) stain for fungus, tissue Gram's stain for bacteria's, and acid-fast stains for tubercular bacilli were done. IHC panels were done for CD 20 (B-cells), CD-3 (T-cells), CD-45 (Leukocyte common antigen, LCA), BCL-2, CD-138 (Plasma cells), Kappa, Lambda, IgG-4 in tissue, IgG-4 in serum, etc. IHCs were done using kit methods (standardized) and adequate controls were taken for each sample. Results: 93 cases of nonspecific orbital inflammation were reported out of 1,467 specimens. Orbital pseudotumors (idiopathic orbital inflammatory disease, IOID) were seen in 27 cases (sclerosing variety-6); benign lymphoid hyperplasia in two cases; reactive lymphoid hyperplasia in 10 cases; atypical plasma lymphoproliferative reactive (polyclonal immunophenotypically, IgG4 negative) lesions in four cases; IgG-4 related disease in one case; nonspecific inflammatory reactions (conjunctiva, sclera, and lid) in 49 cases. In all the diagnoses, infections and lymphomas were excluded. Conclusion: Biopsy supported study on nonspecific orbital inflammation was important to know the pattern.

IgG4-related ophthalmic disease. Part I: background and pathology.

PURPOSE: To review the current state of knowledge of IgG4-related ophthalmic disease (IgG4-ROD). METHODS: A review of the literature and personal experience of the authors. RESULTS: IgG4-related disease is a recently recognized fibroinflammatory disorder that may affect 1 or more organs. It is characterized by lymphoplasmacytic infiltrates with large numbers of IgG4 positive plasma cells, storiform fibrosis, obliterative phlebitis, and eosinophil infiltration as well as peripheral eosinophilia, and in some cases, elevated serum levels of IgG4. These features are not always seen, and the diagnosis should be made by integrating clinical, imaging, and histopathological data, with reference to recently defined diagnostic criteria. IgG4-ROD forms a significant proportion of what has previously been labeled "idiopathic orbital inflammation" or reactive lymphoid hyperplasia. Orbital disease may occur alone, at the same time as disease elsewhere, or metachronously with systemic disease. Although almost any ocular adnexal tissue may be affected, there are several more common recognizable patterns of IgG4-ROD: 1) sclerosing dacryoadenitis; 2) enlargement of orbital nerves (most commonly the infraorbital nerve) associated with orbital myositis and lacrimal gland disease, often in combination with paranasal sinus disease, eosinophilia, and systemic involvement; and 3) sclerosing orbital inflammation. CONCLUSIONS: Patients presenting with orbital inflammatory lesions should have biopsies obtained whenever possible. The examining pathologist should routinely look for features of IgG4-ROD, and if found, the patient should be investigated for other organ involvement. Early treatment may prevent destructive changes in affected tissues.

Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review.

BACKGROUND: Involvement of orbital structures by immunoglobulin G4-related disease (IgG4-RD) is not uncommon. We conducted this study to evaluate the clinicopathologic features of orbital IgG4-RD. MATERIAL/METHODS: This was a retrospective, clinicopathologic study. Clinical records, light microscopic features, results of immunostaining with IgG & IgG4 and laboratory findings were reviewed in 16 patients diagnosed with orbital IgG4-RD. RESULTS: Eleven patients had a bilateral disease, and the lacrimal gland was involved in 14. Dense sclerosis, plasma cell aggregates and dense lymphoplasmacytic infiltrate were seen in all patients. Serum IgG4 titre was elevated in 12 patients. Nine patients responded completely to glucocorticoid treatment. Five patients had a relapse on discontinuation of treatment. CONCLUSION: Orbital IgG4-RD is a distinct clinicopathologic entity requiring increased awareness and needs to be differentiated from other orbital lymphoproliferative lesions.

Medial rectus muscle myositis as an atypical presentation of mucosa-associated lymphatic tissue lymphoma: a case report.

Here we describe the rare case of a 55-year-old man with medial rectus muscle myositis as an atypical presentation of non-Hodgkin B-cell mucosa-associated lymphoma (MALT). Pathology and immunohistochemistry of the affected muscle confirmed the diagnosis of a neoplasm. The primary etiology of orbital myositis is Graves' ophthalmopathy, but several other diseases may cause this clinical presentation. Therefore, the neoplastic causes must be eliminated from the differential diagnoses. non-Hodgkin B-cell mucosa-associated lymphoma is the most common histological type of lymphoma in the orbit, with the conjunctiva and lacrimal glands being the most commonly affected sites. However, it may also present in atypical forms involving others sites and tissues.

Medial rectus muscle myositis as an atypical presentation of mucosa-associated lymphatic tissue lymphoma: a case report / Miosite do músculo reto medial como forma atípica de apresentação de linfoma tipo MALT: relato de caso

Here we describe the rare case of a 55-year-old man with medial rectus muscle myositis as an atypical presentation of non-Hodgkin B-cell mucosa-associated lymphoma (MALT). Pathology and immunohistochemistry of the affected muscle confirmed the diagnosis of a neoplasm. The primary etiology of orbital myositis is Graves' ophthalmopathy, but several other diseases may cause this clinical presentation. Therefore, the neoplastic causes must be eliminated from the differential diagnoses. non-Hodgkin B-cell mucosa-associated lymphoma is the most common histological type of lymphoma in the orbit, with the conjunctiva and lacrimal glands being the most commonly affected sites. However, it may also present in atypical forms involving others sites and tissues.

Descrevemos um raro caso de miosite do músculo reto medial como forma atípica de apresentação de linfoma não-Hodgkin de células B tipo MALT. A anatomia patológica e imuno-histoquímica do músculo afetado confirmaram o diagnóstico definitivo do caráter neoplásico da doença. As miosites orbitárias têm como principal etiologia a oftalmopatia de Graves, porém diversas outras causas podem apresentar-se dessa forma. Sendo assim, as causas neoplásicas devem ser descartadas. O linfoma não-Hodgkin de células B tipo MALT é o tipo histológico mais comum de linfoma orbitário, as regiões mais frequentemente acometidas são a conjuntiva e glândula lacrimal. No entanto, pode apresentar-se com formas clínicas atípicas, acometendo outras regiões e tecidos.

Idiopathic orbital myositis.

PURPOSE: There is a paucity of reliable data and limited experience on the clinical features and therapeutic outcomes of orbital myositis. The purpose of this study was to collate data on the clinical features, imaging, diagnosis, and therapeutic effects of case reports from patients with myositis of idiopathic orbital inflammation pseudotumor in a tertiary eye hospital in China. METHODS: A retrospective study was performed on the records of 44 Chinese patients with orbital myositis. Data were obtained from the period of January 1, 2000, to August 31, 2010, from patients treated at the Eye Hospital of the Zhongshan Ophthalmic Center at Sun Yat-sen University, Guangzhou, China. RESULTS: Twenty-five patients were women and 19 men. The mean age was 39.1 years (range, 11-77 years). Right eyes were involved in 18 cases, left in 17 cases, and both eyes in 9 cases. Dysfunctions of the affected muscles included pure paretic (20.5%), pure restrictive (45.5%), or mixed paretic-restrictive (34.1%). The ratio of acute to subacute stage was 1:3. The rank order of affected muscles was as follows: superior rectus (29.1%), lateral rectus (25.6%), medial rectus (24.4%), inferior rectus (19.8%), and superior oblique (1.16%). The proportion of single muscle involvement was 37.5%, and tendon involvement was seen in 40.9% of the muscles. All patients were treated with systemic corticosteroids (prednisone or dexamethasone). Full recovery was achieved in 38.6% of patients, whereas 59.1% achieved partial recovery with an average of 6.4 recurrences (range, 2.0-8.0 recurrences). Recurrences occurred in 81.8% of the patients. CONCLUSIONS: Orbital myositis occurs in multiple clinical manifestations and may be recurrent. Imaging is an important technique for use in diagnosis. Systemic corticosteroid represents an effective approach for treatment.

Orbital masses: the usefulness of diffusion-weighted imaging in lesion categorization.

INTRODUCTION: Diffusion-weighted imaging (DWI) produces contrast among different kinds of tissues according to their diffusibility characteristics. The purpose of our study was to evaluate the role of DWI including measurement of apparent diffusion coefficient (ADC) values in recognizing benignancy or malignancy of orbital masses. METHODS: A total of 39 orbital masses were evaluated visually for signal characteristics on DWI and ADC maps. ADC values were calculated for each lesion. Visual signal characteristics were compared using the Fisher exact test. Receiver operating characteristic (ROC) analysis was carried out to determine sensitivity and specificity for distinguishing malignant from benign lesions using ADC values. The Mann-Whitney U test was applied to compare the ADC values between orbital lymphomas and idiopathic orbital inflammatory (IOI) lesions, and between optic nerve sheath meningiomas and gliomas. RESULTS: Visual assessment revealed no significant difference between benign and malignant lesions on DWI (p-value = 0.66). However, visual assessment of ADC maps revealed a statistically significant (p-value ≤ 0.0001) between benign and malignant lesions. ROC analysis showed a sensitivity of 83.33 % and a specificity of 85.71 % when using an optimal cut off ADC value of 0.84 × 10(-3) mm(2)/s for differentiating malignant from benign lesions. Significant differences in mean ADC values were observed between lymphomas and IOI lesions (p-value = 0.05), and between optic nerve sheath meningiomas and gliomas (p-value = 0.03). CONCLUSION: DWI is useful for differentiating malignant and benign orbital tumors if accompanied by visual assessment of ADC maps and ADC value calculations.

Efeitos da injeção intraorbitária de carboximetilcelulose 6.0% em coelhos: análise histológica e da mecânica ocular / Effects of intraorbital injection of 6.0% carboxymethylcellulose in rabbits: histological and ocular mechanics evaluation

OBJETIVO: Avaliar se a carboximetilcelulose 6,0% é capaz de atuar como modificadora do sistema oculomotor de coelhos por meio de forças de ação viscoelástica, a durabilidade de seu eventual efeito e reações teciduais à aplicação. MÉTODOS: Foram utilizados 25 coelhos da raça Nova Zelândia, divididos em dois grupos experimentais: um tratado com injeção peribulbar de carboximetilcelulose (CMC) 6,0 por cento e um controle que foi submetido à injeção peribulbar de 3,0 cc de soro fisiológico. No grupo tratado com CMC, variou-se o volume total injetado, obtendo-se, assim, quatro subgrupos (1,0, 1,5, 2,0 e 3,0 cc). Foram realizadas medidas da força necessária para promover movimentos tangenciais de adução, avaliação clínica e medidas da pressão intraocular antes, imediatamente após a injeção da substância e no 7º, 30º e 60º dia pós-operatório. A eutanásia dos animais foi realizada no 60º dia pós-operatório para análise histológica dos tecidos perioculares. RESULTADOS: A força média encontrada, 60 dias após a injeção da CMC 6,0 por cento, foi menor no subgrupo tratado 1,0 e maior nos subgrupos tratados 1,5, 2,0 e 3,0 relativamente à força antes da injeção. Nos subgrupos em que houve aumento da força, a análise histológica revelou processo inflamatório do tipo histiocitário com formação de fibrose e a presença da CMC nos tecidos perioculares. CONCLUSÕES: A carboximetilcelulose 6,0 por cento atuou como modificadora do sistema oculomotor de coelhos, podendo facilitar ou dificultar movimentos. Não foi possível concluir se o aumento da força deveu-se apenas ao processo inflamatório ou à soma de inflamação com um possível atrito viscoso provocado pela CMC.

PURPOSE: To determine the effects of 6.0% carboxymethylcellulose (CMC) in modifying the oculomotor system of rabbits by means of viscoelastic action forces, the durability of this effect and possible inflamatory reaction. METHODS: Twenty-five New Zealand rabbits were divided into two experimental groups: one treated with a peribulbar injection of 6.0 percent CMC and a control group submitted to peribulbar injection of 3.0 cc of physiological saline. The group submitted to peribulbar CMC injection was divided into four subgroups respectively receiving the following volumes: 1.0, 1.5, 2.0 and 3.0 cc. The force needed to promote tangential adduction dislocations, external ophthalmologic signs and intraocular pressure were evaluated. The animals were sacrificed on the 60th day after the injection for histological analysis. RESULTS: The mean force detected 60 days after the injection of 6.0 percent CMC was lower in the 1.0 treated subgroup and higher in the 1.5, 2.0 and 3.0 treated subgroups relative to the force before injection. Histological analysis revealed histiocytic infiltration with fibrosis in the subgroups in which there was an increase in force and the presence of CMC in periocular tissues. CONCLUSIONS: 6.0 percent CMC acted as a modifier of the oculomotor system of rabbits, facilitating or impairing movements. It was not possible to conclude whether the increase in force occurred as a consequence of the inflammatory process alone or of the sum of inflammation and a possible viscous attrition provoked by CMC.

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

PURPOSE: We report the successful use of infliximab in a young patient with idiopathic sclerosing orbital inflammation (myositis). METHODS: We describe the patient's medical records and review the literature. RESULTS: 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. CONCLUSIONS: Infliximab and methotrexate might be considered in patients with idiopathic sclerosing orbital inflammation (myositis).