A Case of Mikulicz's Disease, IgG4-related Plasmacytic Syndrome, Successfully Treated by Corticosteroid and Mizoribine, Followed by Mizoribine Alone

Nanke, Yuki; Kobashigawa, Tsuyoshi; Yago, Toru; Kamatani, Naoyuki; Kotake, Shigeru

doi:10.2169/internalmedicine.49.3101

Cited by 21 publications

(11 citation statements)

References 14 publications

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“…Lymphadenopathy is also observed. However, reports regarding lymph node pathology of patients with IgG4-related sclerosing disease are limited, as evidenced by Table 2 [4,5,9]. In our study, we clinicopathologically reviewed three cases of IgG4-related lymphadenopathy.…”

Section: Discussionmentioning

confidence: 97%

Lymphadenopathy of IgG4-related sclerosing disease: three case reports and review of literature

et al. 2010

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Immunoglobulin (Ig) G4-related sclerosing disease is a recently described syndrome characterized by lymphoplasmacytic infiltration of exocrine glands or extranodal tissues and elevated serum IgG4. We report three cases of lymphadenopathy secondary to IgG4-related sclerosing disease. Histologic features of involved lymph nodes included interfollicular immunoblasts and plasma cells, similar to Castleman's disease. The percentage of IgG4-/IgG-positive plasma cells in the three patients was markedly elevated (30, 50, and 60%). Administration of prednisolone led to remission in every case. One of three cases was consulted to our hospital due to suspected diagnosis of angioimmunoblastic T cell lymphoma (AITL). The case demonstrates many clinical and pathologic similarities between IgG4-related sclerosing disease and AITL. Pathological similarities between AITL and the lymphoplasmacytic subtype of IgG4-sclerosing disease have recently been reported. It is important to accurately diagnose IgG4-related lymphadenopathy given its ready response to steroid therapy and the potential for misdiagnosing lymphoma on clinical grounds.

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Section: Discussionmentioning

confidence: 97%

Lymphadenopathy of IgG4-related sclerosing disease: three case reports and review of literature

et al. 2010

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“…In addition, maintenance therapy with steroid medication needs to be investigated. Recently, mizoribine has been used for IgG4‐related sclerosing disease and has proven to be effective for reducing symptoms and serum IgG4 levels 20. Therefore, further studies are required to more fully characterize IgG4‐related diseases in more cases and to clarify the long‐term outcomes.…”

Section: Discussionmentioning

confidence: 99%

Comment on a prospective evaluation of computerized tomographic (CT) scanning as a screening modality for esophageal varices

et al. 2008

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Comment on a Prospective Evaluation of Computerized Tomographic (CT) Scanning as a Screening Modality for Esophageal VaricesTo the Editor:We read with interest the article by Perri et al. published in a recent issue of HEPATOLOGY. 1 Perri et al. report that abdominal computed tomography (CT) performed as the initial screening test for esophageal varices could be cost-effective. The total effective radiation dose for CT was 15 mSv, slightly highly than that for an abdominal and pelvic CT scan. We know that the effective dose, expressed in sieverts, allows for a rough comparison between different CT scenarios but provides only an approximation of the true risk.According to a report by the U.S. National Academy of Sciences, a single population dose of 10 mSv is associated with a lifetime risk of a solid cancer or leukemia of 1 in 2000. 2 The radiation dose is particularly problematic when patients have multiple CT scans, as requested if no varices are identified by CT scan, because screening for varices should then be repeated in 2-3 years according to current recommendations.Although the individual estimates are small, the concern about the risks from CT is related to the rapid increase in its use, and it has been estimated that about 1.5%-2.0% of all cancers in the United States may be attributable to radiation from CT studies. 3 No largescale epidemiologic studies of the cancer risks associated with CT scans have been reported, but one such study is just beginning. 4 The most effective way to reduce the population dose from CT is simply to decrease the number of CT studies that are prescribed, especially if we have a simple and effective method such as gastroscopy.Perri et al. should at least acknowledge these concerns.

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“…Other drugs reported to be effective include azathioprine, methotrexate, mizoribine, 38 mycophenolate mofetil, cyclophosphamide, cyclosporine, bortezomib, 39 and rituximab. [40][41][42][43][44] However, their relative effectiveness and appropriateness have not yet been established.…”

Section: Other Immunomodulatory Agentsmentioning

confidence: 99%

IgG4-Related Disease: Diagnostic Methods and Therapeutic Strategies in Japan

Yasukawa

Shimizu

Nakamura

et al. 2014

J Clin Exp Hematopathol

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This review describes methods utilized in Japan to diagnose and treat patients with IgG4-related disease. A diagnosis of IgG4-related disease is based on elevated serum IgG4 concentration and an increased number of IgG4 + plasma cells. Differentiating IgG4-related disease from other disorders, especially malignancy, is quite important. Consensus treatment in Japan consists of an initial dose of prednisolone at 0.5-0.6 mg/kg/day, followed by careful and gradual dose reduction. Most patients require maintenance treatment at 5 to 10 mg/day. Patients refractory to glucocorticoids are either truly refractory or have been misdiagnosed, therefore requiring reassessment. 〔J Clin Exp Hematop 54(2) : 95-101, 2014〕

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A Case of Mikulicz's Disease, IgG4-related Plasmacytic Syndrome, Successfully Treated by Corticosteroid and Mizoribine, Followed by Mizoribine Alone

Cited by 21 publications

References 14 publications

Lymphadenopathy of IgG4-related sclerosing disease: three case reports and review of literature

Lymphadenopathy of IgG4-related sclerosing disease: three case reports and review of literature

Comment on a prospective evaluation of computerized tomographic (CT) scanning as a screening modality for esophageal varices

IgG4-Related Disease: Diagnostic Methods and Therapeutic Strategies in Japan

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