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BACKGROUND/AIMS: This study investigated the clinical and pathological features of immunoglobulin G4 (IgG4)-related ophthalmic disease. To clarify the features, we compared IgG4-related ophthalmic disease and orbital inflammatory pseudotumor. METHODS: We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis. Immunohistochemical staining of pathological specimens for IgG and IgG4 was performed. Finally, six of IgG4-related ophthalmic disease patient and 10 of orbital inf lammatory pseudotumor patient were analyzed. RESULTS: The IgG4-related ophthalmic disease group had more IgG4-positive plasma cells and a higher IgG4/IgG plasma cell ratio than the orbital inflammatory pseudotumor group. Collagenous fibrosis and lacrimal gland involvement were significantly more frequent in the IgG4-related ophthalmic disease group. Dense lymphocyte infiltration, obliterative phlebitis, and bilateral lesions were more frequent in IgG4-related ophthalmic disease, but the differences were not significant. The recurrence-free period was shorter in the IgG4-related ophthalmic disease group (p = 0.035). CONCLUSIONS: The location of the lesion (lacrimal gland), count and ratio of IgG4-positive plasma cells, and collagenous fibrosis aid the diagnosis of IgG4-related ophthalmic disease in patients with idiopathic orbital mass-like lesions. In addition, maintenance therapy should be considered in patients with IgG4-related ophthalmic disease to prevent recurrence.
Subject(s)
Humans , Biopsy , Collagen , Diagnosis , Fibrosis , Immunoglobulin G , Immunoglobulins , Lacrimal Apparatus , Lymphocytes , Medical Records , Orbit , Orbital Pseudotumor , Phlebitis , Plasma Cells , Recurrence , Retrospective StudiesABSTRACT
@#IgG4-RD is a newly recognized chronic and systemic disease. IgG4-positive plasma cells play a part in the occurrence and development of this disease. “IgG4-related ophthalmic disease(ROD)” was proposed for the involvement of eyes in general. The clinical manifestations of IgG4-ROD are various due to different involved parts and have unique pathologic features compared with other organs. With a better understanding on this disease in recent years, diagnosis and treatment rate has been improved. In this paper, we will discuss the definition, epidemiology, pathogenesis, clinicopathological features, diagnosis and differential diagnosis, serum biochemical indices, imaging examinations and treatment about IgG4-ROD.
ABSTRACT
PURPOSE: To report a case of immunoglobulin G4 (IgG4)-related ophthalmic disease associated with adult xanthogranulomatous disease. CASE SUMMARY: A 38-year-old male with a history of cholecystectomy visited our clinic for bilateral periorbital swelling. Histopathology of the orbital biopsy showed diffuse infiltration of foamy histiocytes with Touton giant cells and lymphoid follicles, with a diagnosis of adult-onset xanthogranuloma. After excisional biopsy, he was treated with azathioprine and prednisolone. Four years after treatment, he again visited the clinic due to bilateral, yellowish eyelid masses. Serological examinations were all nonspecific findings, except for elevation of IgG and IgG4 levels. Magnetic resonance imaging showed bilateral symmetric soft tissue enlargement with slightly heterogeneous T1/T2 isosignal intensity, with contrast enhancement at the superolateral aspect of extraconal spaces. Excisional biopsy and blepharoplasty were performed. Immunohistochemical sections showed that the IgG4+/IgG plasma cell ratio was 10–20% and the IgG4 plasma cell count was 22/high power field (HPF). His past sections of 2013 from the pathology department were again stained and showed that the IgG4+/IgG plasma cell ratio was 40–50% and the IgG4 plasma cell count was 59/HPF. Thus, he was definitely diagnosed with IgG4-related ophthalmic disease. CONCLUSIONS: If there is recurrent eyelid swelling, IgG4-related ophthalmic disease should be considered as a differential diagnosis. And the patient with adult xanthogranulomatous disease can be diagnosed with IgG4-related ophthalmic disease.
Subject(s)
Adult , Humans , Male , Azathioprine , Biopsy , Blepharoplasty , Cholecystectomy , Diagnosis , Diagnosis, Differential , Eyelids , Giant Cells , Histiocytes , Immunoglobulin G , Immunoglobulins , Magnetic Resonance Imaging , Orbit , Pathology , Plasma Cells , PrednisoloneABSTRACT
Immunoglobulin G4 (IgG4)-related disease is a recently recognized entity characterized by abundant tissue infiltration by IgG4-positive plasma cells and high serum levels of IgG4. IgG4 disease can also affect the lacrimal glands and periocular tissues and usually manifest painless eyelid or periocular tissue swelling. However, IgG4 related ophthalmic disease rarely cause diplopia. We report a case of diplopia IgG4 related ophthalmic disease presenting as diplopia and headache.
Subject(s)
Diplopia , Eyelids , Headache , Immunoglobulin G , Immunoglobulins , Lacrimal Apparatus , Plasma CellsABSTRACT
·Adult orbital xanthogranulomatous disease is a group of rare orbital and ocular adnexal disorders, which is classified as class II non - Langerhans histiocytic proliferations. This disease can be classified into 4 subtypes based mainly on systemic involvement: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease and adult-onset asthma and periocular xanthogranuloma. Diagnosis depends on its characteristic clinical manifestations and pathologic features. Therapeutic approaches are derived from anecdotal evidences, which include corticosteroid, immunosuppressive agent, surgical debulking and chemotherapy. The management of this disease varies with different subtypes, as well as associated systemic presentations. This review summarizes advances of etiopathogenesis, clinical features, diagnosis and treatment.
ABSTRACT
PURPOSE: In the present study, a case of IgG4-related ophthalmic disease which met the 2015 IgG4-related ophthalmic disease diagnostic criteria is reported and literature review performed. CASE SUMMARY: A 51-year-old female presented with both upper eyelid swelling, redness, and a palpable mass. Eye movements were normal and exophthalmos was not observed. Facial computed tomography showed both lacrimal gland hypertrophy. The patient underwent left anterior orbitotomy with incisional biopsy. Immunostained biopsy showed the ratio of IgG4+ to IgG+ cells was 50% and the mean number of IgG4-positive plasma cells was approximately 150 per high-power field. Hematological examination showed elevated serum IgG4 concentrations of 6,930 mg/dL, however, other organs were not involved. The patient satisfied the diagnostic criteria for IgG4-related ophthalmic disease. The patient was given an oral steroid and immunosuppressant and the symptoms improved. CONCLUSIONS: IgG4-related ophthalmic disease is currently receiving increased attention. The authors of the present study report a case of IgG4-related ophthalmic disease which met the 2015 IgG4-related ophthalmic disease diagnostic criteria.