H&E staining at low magnification (A 100) and high magnification (B 200) showing infiltration of plasma cells surrounding the mantle zones and a pattern of concentric deposits with lymphoid cells. == Introduction == The pathogenesis of IgG4-related dacryoadenitis and Mikuliczs disease is different from that of Sjgren syndrome.1However, the precise pathological definition of IgG4-related dacryoadenitis is still debated because there are other diseases with similar clinicopathological features, eg, Castleman disease.2We report a case of chronic sclerosing bilateral dacryoadenitis with high level of the serum IgG4 that had been diagnosed with Castleman disease from the histopathological findings of a submandibular gland biopsy. == Case report == A 79-year-old man first noticed a swelling of his lower left jaw 8 years earlier. He was diagnosed with Castleman disease by histopathological examinations of a specimen from a swollen submandibular gland 6 years earlier. Since then, the size of the swollen gland had not changed and he had no symptoms or signs of systemic inflammation. He developed diplopia a year ago, and CT scans showed swelling of the lacrimal glands bilaterally, and he was referred to our hospital for further examinations. Our examination showed that his visual acuities were slightly reduced to 0.8 OD, 0.5 OS because of moderate cataracts. The intraocular pressures and fundus findings were within normal limits. His left eye was shifted to downward because of a swollen lacrimal gland. The results of the Schirmer test were within normal limits, but MRI findings showed bilateral lacrimal gland swelling (Figure 1). He had no visual field defects but his eye movements were slightly restricted. He had no histories of systemic symptoms such as weight loss, skin legions, HIV infection, or recent vaccinations. == Figure 1. == T2 MRI images of a patient with swollen lacrimal glands. The MRI images were obtained at the first visit to our hospital. The MRI findings indicate extreme swelling of both lacrimal glands. He underwent partial dacryoadenectomy on the right side because it was easier to approach than the left side. Histopathological examination of the lacrimal gland biopsy showed inflammatory cells that resembled the histology of Castleman disease (Figure 2). However, some histopathological findings did not agree with the histology of Castleman disease (Figure 2). His blood tests showed high levels of interleukin-6 (IL-6; 5.1 pg/mL), IL-2 receptor (1168 U/mL), and IgG (2790 mg/dL), which are consistent with Castleman disease. However, the serum level of IgG4 Anemoside A3 was also high (695 mg/dL). Immunohistochemical analysis showed that the specimen was not positive for IgG4 (data not shown). The final diagnosis was that our patient had chronic sclerosing dacryoadenitis with high level of the serum IgG4 in addition to Castleman disease. == Figure 2. == Histopathological findings in a specimen of the right lacrimal gland. H&E staining at low magnification (A 100) and high magnification (B 200) showing infiltration of plasma cells surrounding the mantle zones and a pattern of concentric deposits with lymphoid Anemoside A3 cells. These are pathognomic of Castleman disease. The infiltrated cells surrounding the mantle zones were CD138 (C 100), Igkappa (E 100), Iglambda (F 100), and CD79a positive (D 100). However, compared to plasma cell type of Castleman disease, fibrosis in the interstitial tissue was relatively greater. Compared to hyaline-vascular type of Castleman disease, vascularization in the intrafollicular area was relatively poor. The development of mantle zones and the pattern of concentric deposits with infiltrating cells are not of typical of Castleman disease. We finally decided Anemoside A3 these tissues resemble reactive lesions rather than Castleman disease. He underwent oral Rabbit Polyclonal to HUCE1 steroid therapy (30 mg) and the swollen lacrimal glands and his double vision were significantly improved (Figure 3). His last examination 6 months after the steroid therapy showed that his lacrimal glands were not changed. == Figure 3. == HESS charts and MRI findings before and after oral steroid administration. Both HESS charts and MRI findings were significantly improved after oral steroid administration. Anemoside A3 Six months after the treatment, no recurrence was observed. The effect of the oral steroid treatment was consistent with the histopathological findings of the specimen from. Anemoside A3
