RESUMEN
A 38-year-old female patient presented with a painful swelling in the lateral part of the upper eyelid, a diffuse scleritis and slight hypoglobus of the right eye. An orbital biopsy showed a fibrotic idiopathic orbital inflammation (IOI) with, on immunohistochemical staining, an increased number of IgG4-positive plasma cells scored as >200 per high-power field, with IgG4/IgG ratio >0.50, indicating orbital IgG4 related autoimmune disease. On treatment with oral prednisone and azathioprine the symptoms resolved within 6 months. Twenty years prior, the patient had been diagnosed with an IOI of at the same side, for which at that time a biopsy had been taken similarly. Reclassification of the previous biopsy specimen with immunohistological staining also showed evidence of orbital IgG4 related disease. To our knowledge this is the first report of a biopsy-proven unilateral IgG4-related orbitopathy that recurred after 20 years.
Asunto(s)
Hipergammaglobulinemia/diagnóstico , Inmunoglobulina G/sangre , Seudotumor Orbitario/diagnóstico , Administración Oral , Adulto , Azatioprina/uso terapéutico , Biopsia , Quimioterapia Combinada , Femenino , Glucocorticoides/uso terapéutico , Humanos , Hipergammaglobulinemia/tratamiento farmacológico , Hipergammaglobulinemia/inmunología , Inmunosupresores/uso terapéutico , Seudotumor Orbitario/tratamiento farmacológico , Seudotumor Orbitario/inmunología , Prednisona/uso terapéutico , Recurrencia , Factores de Tiempo , Tomografía Computarizada por Rayos XRESUMEN
PURPOSE: To determine the relation of orbital xanthogranuloma with IgG4-related disease. METHODS: Retrospective consecutive case series over a period of 25 years. We searched our charts for histologically confirmed orbital xanthogranuloma. Patient files were reviewed for clinical and follow up data including presence or absence of systemic non-ophthalmic manifestations of IgG4 related disease. Slides were re-examined and histopathological classification was re-assessed. Sixteen cases of orbital xanthogranuloma were evaluated. Immunohistochemical stains for IgG and IgG4 were performed. Positive immunohistochemical staining required increased IgG4-positive plasma cells in the involved tissues scored as >50 per high-power field, with IgG4/IgG ratio >0.40. RESULTS: According to the criteria described above 8/16 (50%) cases showed increased numbers of IgG4-positive plasma cells in the specimens. Two of these patients may have had signs of systemic disease. CONCLUSION: Raised numbers of IgG4-positive plasma cells are a common finding in histopathological specimens of xanthogranulomatous disease of the orbit and are often not indicative for IgG4 related systemic disease.