Project description:The aim of the present study was to observe the histopathological changes of immunoglobulin G4-related orbital diseases (IgG4-RODs), summarize the clinical manifestations and imaging features of the IgG4-RODs of the eyelids and explore the early diagnosis of IgG4-RODs. Between June 2011 and May 2015, 23 patients with non-specific orbital inflammation in the Department of Ophthalmology at the First Central Hospital of Tianjin were recruited. The serum IgG4 titer in 9 patients ranged from 4.58 to 46.70 g/l (reference value, 0.03-2.01 g/l), with an average value of 21.93±2.18 g/l. Notably, the degree of increase in the 9 patients with IgG4-RODs was different, but all were >1.35 g/l. A total of 6 cases of infraorbital nerve thickening were observed. In addition, there were 3 cases of extraocular muscle thickening and 1 patient with IgG4-ROD had an orbital tissue lesion extending along the inferior temporal septum to the left pterygopalatine fossa, with left sacral fissure widening and involvement of the left maxillary sinus. The study revealed that the thickening of the inferior orbital nerve may be a characteristic of IgG4-ROD. Therefore, on the basis of biopsy and serological examination in the clinic, early diagnosis can be combined with imaging examination, clinical manifestation and laboratory examination, so as to reduce misdiagnosis and missed diagnosis.
Project description:A 23-year-old man with recurrent acute myeloid leukemia (AML) underwent successful reinduction and was judged posttherapy to be in complete remission. Soon thereafter, he complained of pain in his left buttock radiating into his left posterior thigh. Neurologic examination was unremarkable. Radiographic evaluation demonstrated a left S2 lesion suggestive of a nerve sheath tumor (figure 1). An open biopsy was performed that revealed a chloroma pathologically (figure 2), sometimes referred to as a myeloid sarcoma.(1,2) Most chloromas are found in patients with recurrent AML and are overwhelmingly intracranial.(1) Infrequently, chloromas are paraspinal, and in this location present with epidural spinal cord compression.(2) Intraspinal invasion by a chloroma is rare. Systemic evaluation confirmed recurrent AML, for which he was successfully treated with reinduction and whole-body irradiation followed by an allogeneic transplant. He is currently disease-free and neurologically asymptomatic 1 year posttransplant.
Project description:ObjectiveIgG4-related disease is an emerging clinical entity which frequently involves tissue within the orbit. In order to appreciate the implications of IgG4 immunostaining, we analyzed gene expression and the prevalence of IgG4- immunostaining among subjects with orbital inflammatory diseases.MethodsWe organized an international consortium to collect orbital biopsies from 108 subjects including 22 with no known orbital disease, 42 with nonspecific orbital inflammatory disease (NSOI), 26 with thyroid eye disease (TED), 12 with sarcoidosis, and 6 with granulomatosis with polyangiitis (GPA). Lacrimal gland and orbital adipose tissue biopsies were immunostained for IgG4 or IgG secreting plasma cells. RNA transcripts were quantified by Affymetrix arrays.ResultsNone of the healthy controls or subjects with TED had substantial IgG4 staining. Among the 63 others, the prevalence of significant IgG4-immunostaining ranged from 11 to 39% depending on the definition for significant. IgG4 staining was detectable in the majority of tissues from subjects with GPA and less commonly in tissue from subjects with sarcoidosis or NSOI. The detection of IgG4+ cells correlated with inflammation in the lacrimal gland based on histology. IgG4 staining tissue expressed an increase in transcripts associated with inflammation, especially B cell-related genes. Functional annotation analysis confirmed this.ConclusionIgG4+ plasma cells are common in orbital tissue from patients with sarcoidosis, GPA, or NSOI. Even using the low threshold of 10 IgG4+ cells/high powered field, IgG4 staining correlates with increased inflammation in the lacrimal gland based on histology and gene expression.