A case report of Mikulicz's disease.

Mikulicz's disease is a unique condition involving the enlargement of the lacrimal and salivary glands, similar to that observed in Sjogren's syndrome; however, Mikulicz's disease is clinically characterized by infrequent autoimmune reactions and responsiveness to glucocorticoid treatment. The ultrasound features of the lacrimal and salivary glands in patients with IgG4-Mikulicz's disease were characterized by multiple hypoechoic areas of varying sizes within the enlarged glands. IgG4 serum level was also elevated, in contrast to the detection of normal levels in Sjogren's syndrome. In this article, we intended to illustrate a case of Mikulicz's disease with clinical and imaging features.

Preferential M2 macrophages contribute to fibrosis in IgG4-related dacryoadenitis and sialoadenitis, so-called Mikulicz's disease.

IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS) is characterized by bilateral swelling of glandular tissues with extensive fibrosis, and is immunologically considered a Th2-predominant disease. Recent studies reported that alternatively activated (M2) macrophages enhanced Th2 immune responses and fibrosis by production of pro-fibrotic factors (IL-10, IL-13 and CCL18). Therefore, we examined the association between M2 macrophages and fibrosis in submandibular glands from 7 patients with IgG4-DS, 10 patients with chronic sialoadenitis, 10 patients with Sjögren's syndrome, and 10 healthy subjects. The number of M2 macrophages in SMGs from patients with IgG4-DS was also significantly higher than in the other groups. Double immunofluorescence staining showed that IL-10 and CCL18 expression co-localized with M2 macrophage-marker (CD163). Furthermore, the SMG fibrosis score was positively correlated with the frequency of M2 macrophages in only IgG4-DS. These results indicate that IL-10 and CCL18 secreted by preferential M2 macrophages possibly play a key role in the development of severe fibrosis in IgG4-DS.

[IgG4-related systemic disease/systemic IgG4-related disease].

IgG4-related systemic disease/systemic IgG4-related disease has been established as a new systemic disease entity. It is characterized by high serum IgG4 concentrations and abundant IgG4-bearing plasma cell infiltration in the involved organs. The chronic inflammation can attack lacrimal glands, salivary glands, the thyroid, lung, pancreas, kidney, and prostate. The concept includes Mikulicz's disease, Riedel's thyroiditis, pulmonary fibrosis, pulmonary pseudotumor, autoimmune pancreatitis, a part of tubulointerstitial nephritis, and chronic prostatitis. It is important to note that these lesions can occur at different times and sites. So, it is necessary to reconfirm the disease definition and entity in each specialized field. The diagnosis of this disease is confirmed by the above serological and histopathological characteristics. There are clinical diagnostic criteria of Mikulicz's disease (the Japanese Medical Society for Sjögren's Syndrome) and autoimmune pancreatitis (the Japanese Ministry of Health, Labour and Welfare, and the Japan Pancreas Society). They are convenient and useful. Glucocorticoid improves the physical abnormalities, and the initial dose of prednisolone is 30 mg/day, tapered in 5-mg reductions every two weeks. Nevertheless, there are some cases unable to achieve complete remission.

Lacrimal gland function in autoimmune pancreatitis.

OBJECTIVE: Autoimmune pancreatitis (AIP) may be a pancreatic lesion of IgG4-related systemic disease. Lacrimal gland swelling is a rare extrapancreatic lesion of AIP. The aim of the present study was to investigate lacrimal gland function in AIP patients, and to determine changes after steroid therapy. PATIENTS AND METHODS: Schirmer's test and sialochemistry were done prospectively in 11 AIP patients. These tests were also performed after steroid therapy in 7 patients. RESULTS: Dysfunction of tear secretion was found in at least one eye in 7 (64%) patients. The average lower level in both eyes was 4.3+/-1.5 mm in the 7 patients with lacrimal gland dysfunction, which was significantly lower than the 8.2+/-2.4 mm in patients with normal lacrimal gland function (p=0.005). There were no significant differences between the two groups in age at diagnosis of AIP, sex ratio, and the presence of swelling of the lacrimal glands and the salivary glands. Although there was no significant difference, mean serum IgG4 levels and mean salivary Na+ and beta2 microglobulin levels were lower in patients with normal lacrimal gland function. After steroid therapy, lacrimal gland function improved in 3 of 5 patients with impaired lacrimal gland function, though the degree of improvement was not marked compared to the improvement of salivary gland function. CONCLUSION: Lacrimal gland function was frequently impaired in AIP patients, even when no lacrimal gland swelling was observed clinically. Lacrimal gland function impairment appears to be similar to impairment of salivary gland function in AIP patients.