Mikulicz's Disease with Progressively Transformed Germinal Centers-type Immunoglobulin G4-related Lymphadenopathy Mimicking Sjögren's Syndrome

Kim, Hye-Ji; Kim, Jean A; Min, Jun Ki

doi:10.4078/jrd.2015.22.6.395

Cited by 3 publications

(1 citation statement)

References 14 publications

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“…This study also suggested central nervous system involvement such as meningitis as a risk factor of the relapse. Clinical and laboratory features and prognosis of IgG4-RD may vary depending on the involved organ [34][35][36]. Lanzillotta et al reported that patients with head and neck involvement showed a favorable relapse-free outcome at 12 months compared to patients with other organ involvement [37].…”

Section: Plos Onementioning

confidence: 99%

Serum IgG4 level during initial treatment as a predictor of relapse in IgG4-related disease

Choi¹,

Ahn²,

Oh³

et al. 2023

PLoS ONE

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Introduction We aimed to investigate the predictors of relapse in immunoglobulin G4-related disease (IgG4-RD), focusing on the serum IgG4 levels during initial treatment. Methods We retrospectively recruited 57 patients with IgG4-RD who were treated with immunosuppressants and elevated serum IgG4 levels in a tertiary hospital between January 2011 and December 2020. They were followed up for ≥ 6 months after initiation of immunosuppressive therapy. Clinical and laboratory findings including serum IgG4 levels (reference value: 6–121 mg/dL) were compared between relapsed (n = 13) and non-relapsed (n = 44) groups. Multivariate Cox regression analysis was used to assess the predictors for relapse. We performed a Kaplan–Meier analysis with a log-rank test to evaluate the cumulative relapse rate for two years. Results Median serum IgG4 levels at baseline were 321 mg/dL in the relapsed group and 299 mg/dL in the non-relapsed group. Serum IgG4 levels were normalized after six months in five (38.5%) relapsed and 28 (63.6%) non-relapsed patients. In multivariate Cox regression analysis, the normalization of serum IgG4 levels at six months was associated with a lower risk of relapse, with a hazard ratio of 0.232 (p = 0.019). Central nervous system involvement was associated with the relapse, with a hazard ratio of 21.130 (p = 0.015). The cumulative relapse rate for two years was lower in the normal serum IgG4 group at six months than in the elevated serum IgG4 group at six months (p = 0.027). Conclusion Our study suggests that normalization of serum IgG4 levels during immunosuppressive treatment for IgG4-RD independently predicts relapse-free outcomes. Thus, monitoring serum IgG4 levels might be used as a marker of prognosis.

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Section: Plos Onementioning

confidence: 99%

Serum IgG4 level during initial treatment as a predictor of relapse in IgG4-related disease

Choi¹,

Ahn²,

Oh³

et al. 2023

PLoS ONE

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T Cell Roles and Activity in Chronic Sclerosing Sialadenitis as IgG4-Related Disease: Current Concepts in Immunopathogenesis

Ibrahem

2022

Autoimmune Diseases

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IgG4-related disease is a multiorgan immunological fibroinflammatory disorder characterized by lymphoplasmacytic infiltration and fibrosis in multiple organs accompanied by high serum IgG4 levels. The salivary glands are the most common organs involved in this disease. Recently, chronic sclerosing sialadenitis affecting salivary glands, formerly known as Küttner’s tumor, and Mikulicz’s disease have been classified as a class of IgG4-related diseases. The etiopathobiology of IgG4-related disease is not fully understood. It has recently been hypothesized that the inflammatory and fibrotic process and the increased serum IgG4+ levels in IgG4-related disease are the result of an interaction between B cells and T helper cells, suggesting that T cells may play a key role in the pathogenesis of this disease. The aim of this review is to discuss the proposed roles of different T cell subsets in the pathogenesis of IgG4-related disease focusing on their roles in immunopathogenesis of IgG4-related sialadenitis.

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Mikulicz Disease in Males: A Comprehensive Review

Devi Yerra,

Margaret Puvvada,

Kumar Mugada

et al. 2023

AJPS

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IgG4-related disease (IgG4-RD) is an intricate autoimmune disorder that have an effect on multiple organs in the body, including the lachrymal and salivary glands, liver, biliary duct, thyroid, pancreas, gastrointestinal tract, kidney, aorta, prostate and retroperitoneum. Mikulicz disease, a subtype of IgG4-RD, specifically targets the lachrymal and salivary glands, leading to glandular swelling, xerostomia, and xerophthalmia. Diagnosis of Mikulicz disease is established on the presence of proportional and persistent inflammation of at least two pairs of lachrymal, submandibular, and parotid glands, along with exalted serum IgG4 levels and/or marked penetration of IgG4-positive plasmacytes into the affected glandular tissues. Treatment with glucocorticoids has shown to be beneficial in managing IgG4-related MD, but the efficacy of combination therapy in preventing relapse is still a topic of debate. The underlying causes of the disease can vary, but lymphoepithelial sialadenitis is the widespread cause. The disease typically presents in individuals with an average age ranging from 55 to 65 years, and the incidence among individuals under the age of 20 is exceedingly rare. Mikulicz disease is an enigmatic ailment that is still being researched. However, identifying its hallmark symptoms and distinguishing it from other similar conditions is essential for effective diagnosis and treatment. Further research is essential to better comprehend the fundamental causes of the disease and to promote more effective treatment options for patients with Mikulicz disease and other subtypes of IgG4-RD.

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Mikulicz's Disease with Progressively Transformed Germinal Centers-type Immunoglobulin G4-related Lymphadenopathy Mimicking Sjögren's Syndrome

Cited by 3 publications

References 14 publications

Serum IgG4 level during initial treatment as a predictor of relapse in IgG4-related disease

Serum IgG4 level during initial treatment as a predictor of relapse in IgG4-related disease

T Cell Roles and Activity in Chronic Sclerosing Sialadenitis as IgG4-Related Disease: Current Concepts in Immunopathogenesis

Mikulicz Disease in Males: A Comprehensive Review

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