Everyday clinical practice in IgG4-related dacryoadenitis and/or sialadenitis: Results from the SMART database

Yamamoto, Motohisa; Yajima, Hidetaka; Takahashi, Hiroki; Yokoyama, Yoshihiro; Ishigami, Keiichi; Shimizu, Yoshitaka; Tabeya, Tetsuya; Suzuki, Chisako; Naishiro, Yasuyoshi; Takano, Kenichi; Yamashita, Ken; Hashimoto, Masato; Keira, Yoshiko; Honda, Saho; Abe, Takashi; Suzuki, Yasuo; Mukai, Masaya; Himi, Tetsuo; Hasegawa, Tadashi; Imai, Kohzoh; Shinomura, Yasuhisa

doi:10.3109/14397595.2014.950036

Cited by 95 publications

(74 citation statements)

References 20 publications

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“…6,7,17,26,30,32,34,35,39,40 IgG4-related orbital disease has been a popular topic recently, and multiple therapies are being offered to patients, but corticosteroids again remain the preeminent treatment for orbital and systemic IgG4-related disease. [8][9][10]13,16,18,19,24,29,31,[47][48][49] While IgG4-related disease benefits from induction and then slow taper of corticosteroid therapy, the rate of taper is individualized according to patient response. 20 This treatment scheme does not differ from that of steroid therapy for orbital inflammation of unclear etiology.…”

Section: Lack Of Targeted Therapymentioning

confidence: 99%

Orbital inflammation: Corticosteroids first

Glass

Freitag

2016

Survey of Ophthalmology

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Section: Lack Of Targeted Therapymentioning

confidence: 99%

Orbital inflammation: Corticosteroids first

Glass

Freitag

2016

Survey of Ophthalmology

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“…The average age at onset of symptoms is 50-60 years, (37,38) but some patients can show symptoms at a younger age, and the disease can even affect children (39-41).…”

Section: Epidemiologymentioning

confidence: 99%

“…Moreover, patients with IgG4-RD usually do not have constitutional symptoms such as fever, malaise, night sweats, or weight loss (45). Malignancies were reported in 7.4% of the patients with IgG4-RD (37). Extranodal marginal zone B-cell lymphoma may occur in ocular adnexa, salivary glands, or meningeal dura up to 5 years after diagnosis of IgG4-RD (46-48).…”

Section: Clinical Symptomsmentioning

confidence: 99%

IgG4-related disease: A relatively new concept for clinicians

Vasaitis

2016

European Journal of Internal Medicine

102

129

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IgG4-related disease (IgG4-RD) is a recently recognized chronic fibrotic inflammation, which can affect almost every organ, and may come to clinical attention first due to visible organ swelling or organ dysfunction, or is identified incidentally by imaging and specific biopsy.The disorder has an allergic background and is immune-mediated. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in disease progression.About 30-50% of patients are atopic or have mild eosinophilia. IgG4-RD predominantly affects middle-aged male patients. The cornerstones of diagnosis of the disease are compatible clinical features and typical histopathology. Swelling of salivary and lacrimal glands, lymphadenopathy, and type 1 autoimmune pancreatitis (AIP) are the most common manifestations of the disease. However, other tissues and organs, such as retroperitoneum, lung, kidney, aorta, upper airways, thyroid gland, meninges, heart, mesenterium and skin may be involved. Typical histopathology is lymphoplasmacytic infiltration abundant in IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis. Elevated serum IgG4 concentration supports the diagnosis. Characteristic imaging features such as a "capsule-like rim" surrounding the pancreatic lesions is highly specific to type 1 AIP. 18F-fluorodeoxyglucose positron emission tomography/computed tomography enables mapping the sites of inflammation, permits evaluation of the extent of the disease, helps in guiding biopsy decision, and may be used in monitoring response to treatment. Glucocorticoids alone or in combination with B-cell depletion with rituximab induces prompt clinical response to IgG4-RD.This article reviews the current understanding, different clinical manifestations, and approaches to diagnosis and treatment of IgG4-RD. 3Highlights Ø IgG4-related disease is a chronic fibrotic inflammation.Ø The disease affects multiple organs, is progressive, and impairs function.Ø The disorder has an allergic background and is immune-mediated.Ø Compatible clinical signs and typical pathology are the basis for the diagnosis.Ø Glucocorticoids and/or B-cell depletion induces prompt clinical response.Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrotic inflammation, characterized by tissue infiltration by lymphocytes, IgG4-positive plasma cells, simultaneous development of fibrosis, and often, but not always, elevated serum levels of IgG4 (1, 2). The disease can affect multiple organs. Early diagnosis of IgG4-RD is not easy because of the indolent course of the disease with vague, nonspecific symptoms. It may first come to clinical attention as visible organ swelling or detected organ dysfunction due to fibrosis, or is identified incidentally by imaging and specific biopsy when the patient undergoes investigation of suspected tumor disease. Most forms of organ involvement in IgG4-RD, even in the advanced fibrotic stages, respond well to glucocorticoids (3, 4). 4A comprehensive search for all articles in E...

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“…It was found that glucocorticoid has efficacy in all patients with IgG4-RD, but most patients cannot discontinue steroid and require maintenance treatment [2,3]. Furthermore, relapse can easily occur with steroid tapering [4], and there are often patients who need immunosuppressants or biologic agents for disease control [5,6].…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, relapse can easily occur with steroid tapering [4], and there are often patients who need immunosuppressants or biologic agents for disease control [5,6]. However, when we analyzed the patients enrolled in the SMART (Sapporo Medical University and related institutes database for investigation and best treatment for IgG4-RD) registry cohort [2], it was found that they were a fairly diverse group in their clinical features and laboratory data even though they met the comprehensive diagnostic criteria for IgG4-RD [7]. Currently, we are in the era where personalized medicine or, furthermore, precision medicine, presented by former US President Obama [8], should be the aim.…”

Section: Introductionmentioning

confidence: 99%

Predicting therapeutic response in IgG4-related disease based on cluster analysis

Yamamoto

Takano

Kamekura

et al. 2018

Immunological Medicine

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To bring the clinical practice of immunoglobulin (Ig)G4-related disease (IgG4-RD) close to personalized medicine, we classified the patient groups and clarified the therapeutic responses of each group. A total of 147 patients enrolled in our registry were classified into four groups by cluster analysis with the software. The therapeutic responses and prognosis of each group were examined. The cluster analysis classified the subjects into four groups: Cluster 1, patients who presented with prominent hypergammaglobulinemia, elevated levels of serum IgG4, and hypocomplementemia; Cluster 2, patients who presented with eosinophilia, elevated concentrations of serum IgG, IgG4, and IgE, and in whom CRP tended to be positive; Cluster 3, patients with younger onset and serum levels of IgG, IgG4, and IgE and peripheral eosinophil counts lower than the other clusters; and Cluster 4, patients with elder onset and low peripheral eosinophil counts. The amounts of glucocorticoid for maintenance treatment were from 5 to 7 mg/d in all groups, but the amounts were significantly greater in Cluster 1 (patients with hypergammaglobulinemia, elevated levels of serum IgG4, and hypocomplementemia) than in Cluster 4 (elder onset patients, relatively low concentrations of peripheral eosinophils). With regard to the use of immunosuppressants and the relapse rate, there were high frequencies in Cluster 1 and Cluster 3 (younger onset patients who presented with mild elevations of serum IgG and IgG4). On the other hand, Cluster 4 showed a low rate of relapse and often could discontinue steroids. The present results suggest that personalized medicine could be provided in IgG4-RD by classifying patients based on their clinical features. ARTICLE HISTORY

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Everyday clinical practice in IgG4-related dacryoadenitis and/or sialadenitis: Results from the SMART database

Cited by 95 publications

References 20 publications

Orbital inflammation: Corticosteroids first

Orbital inflammation: Corticosteroids first

IgG4-related disease: A relatively new concept for clinicians

Predicting therapeutic response in IgG4-related disease based on cluster analysis

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