IgG4-related Mikulicz's disease associated with thyroiditis: a case report and review of the literature.

PURPOSE: To report an unusual case of IgG4-related Mikulicz's disease associated with thyroiditis. CASE REPORT: We describe a 25-year-old Chinese man who presented with bilateral, painless swellings of the lachrymal glands, parotid glands, and thyroid nodules. The patient underwent left-sided dacryoadenectomy and the diagnosis of IgG4-related Mikulicz's disease was pathologically confirmed. The size of the right-sided lachrymal gland and parotid glands recovered fundamentally after one month of glucocorticoid therapy. CONCLUSION: IgG4-related Mikulicz's disease associated with thyroiditis should be considered in the differential diagnosis of bilateral swellings of lachrymal glands, salivary glands, and thyroid nodules. Surgical excision is recommended in order to treat the tumor and to ensure the pathological diagnosis. Glucocorticoid therapy should be considered in association with surgery after removal.

[Clinical research of immunoglobin 4-related Mikulicz's disease].

OBJECTIVE: To explore the clinical features of immunoglobin (IgG4)-related Mikulicz's disease (MD). METHODS: Since December 2010, a prospective cohort study of IgG4 related disease (IgG4RD) was performed our hospital. A total of 42 IgG4RD patients were recruited along with 18 MD patients. Their clinical, laboratory and histopathological features and response to treatment were analyzed. RESULTS: The 18 MD patients accounted for 42.9% of IgG4RD. There were 10 males and 8 females with a mean age of (48 ± 14) years. The mean follow-up period was 8.18 months. All of them had an involvement of salivary and lacrimal glands. Fifteen cases suffered other organs' damage, including autoimmune pancreatitis (n = 7), sclerosing cholangitis (n = 4) and lymph nodes (n = 6). And 10 patients complained of a history of allergies. All serum levels of IgG4 subclass significantly increased. Histological examinations in 16 patients revealed massive infiltration of lymphocytes and IgG4 positive plasma cells with obvious tissue fibrosis. Glucocorticoid and immunosuppressive therapies were effective for MD. CONCLUSION: As an IgG4-related systemic disease involving predominantly lacrimal and salivary glands without autoantibodies, MD is treated optimally with a combination therapy of glucocorticoid and immunosuppressive drugs.

[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.