Immunoglobulin G4-Related Disease

A spectrum of systemic autoimmune diseases in which IMMUNOGLOBULIN G4 plays a pathophysiologic role. It can affect multiple organs in highly variable presentations, characterized by inflammatory lesions composed of IgG4-positive PLASMA CELLS, further infiltrated by T helper cells (T-LYMPHOCYTES, HELPER-INDUCER) when linked to progressive FIBROSIS and eventual organ damage.

Also Known As:

IgG4 Related Systemic Disease; IgG4-Associated Autoimmune Disease; IgG4-RD; IgG4-Related Disease; Autoimmune Disease, IgG4-Associated; IgG4 Associated Autoimmune Disease; IgG4 Related Disease; IgG4 Related Kidney Disease; IgG4 Related Sclerosing Disease; IgG4-Associated Autoimmune Diseases; IgG4-Related Diseases; IgG4-Related Kidney Diseases; IgG4-Related Sclerosing Diseases; Immunoglobulin G4 Related Disease; Immunoglobulin G4 Related Kidney Disease; Immunoglobulin G4 Related Sclerosing Disease; Immunoglobulin G4-Related Diseases; Kidney Disease, IgG4-Related; Sclerosing Disease, IgG4-Related; IgG4-Related Kidney Disease; IgG4-Related Sclerosing Disease; Immunoglobulin G4-Related Kidney Disease; Immunoglobulin G4-Related Sclerosing Disease

Networked: 1635 relevant articles (139 outcomes, 134 trials/studies)

Relationship Network

Disease Context: Research Results

Immune System Diseases: 4321
- Autoimmune Diseases: 34618
  - Immunoglobulin G4-Related Disease: 1635

Related Diseases

1.	Neoplasms (Cancer)
2.	Autoimmune Pancreatitis
3.	Fibrosis (Cirrhosis)
4.	Retroperitoneal Fibrosis
5.	Lymphadenopathy

Experts

1.	Stone, John H: 61 articles (04/2022 - 01/2012)
2.	Kawano, Mitsuhiro: 49 articles (03/2022 - 11/2010)
3.	Yamamoto, Motohisa: 49 articles (01/2022 - 01/2010)
4.	Okazaki, Kazuichi: 40 articles (06/2022 - 03/2011)
5.	Zhang, Wen: 39 articles (05/2022 - 05/2014)
6.	Takahashi, Hiroki: 39 articles (02/2022 - 01/2010)
7.	Sato, Yasuharu: 35 articles (01/2022 - 12/2009)
8.	Della-Torre, Emanuel: 30 articles (05/2022 - 06/2014)
9.	Deshpande, Vikram: 28 articles (10/2022 - 10/2007)
10.	Saeki, Takako: 27 articles (01/2022 - 11/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Immunoglobulin G4-Related Disease:

1.	GlucocorticoidsIBA 01/01/2021 - "IgG4-RD promptly responds to glucocorticoids and repeated courses are typically used to induce and maintain remission because the disease relapses in most patients. " 10/01/2023 - "While timely diagnosis is particularly important given the efficacy of glucocorticoid treatment for IgG4-RD, accurate recognition can prove a challenge given the overlap between the imaging features of this disease and other entities. " 01/04/2022 - "Although recurrence is very common in IgG4-RD, glucocorticoid is still the first-line treatment for the majority of patients. " 03/02/2023 - "Glucocorticoids are an effective treatment for IgG4-RD if used at a sufficiently high dose, but disease flares are common during or after glucocorticoid tapers and these medications seldom lead to long-term, treatment-free remissions. " 01/01/2017 - "In general, a high-dose glucocorticoid treatment is effective for IgG4-related disease. "
2.	Rituximab (Mabthera)FDA Link 01/01/2023 - "Complete remission of central nervous system manifestations of IgG4-related disease with rituximab - a case report." 06/01/2020 - "Rituximab (RTX) has been shown to be effective in systemic IgG4-related disease (IgG4-RD), but experience with isolated pituitary involvement remains limited. " 08/01/2015 - "Response to: 'Is rituximab effective for IgG4-related disease in the long term? " 08/01/2015 - "Is rituximab effective for IgG4-related disease in the long term? " 09/01/2023 - "The study design ensures a comprehensive evaluation of rituximab's potential efficacy in treating IgG4-RD and its associated clinical characteristics. "
3.	SteroidsIBA 01/01/2020 - "She was diagnosed as having immunoglobulin G4-related disease, and her clinical signs and symptoms improved dramatically after steroid treatment. " 04/06/2023 - "A single-centre retrospective study of factors affecting steroid-free remission of IgG4-related disease conducted in South Korea: a notable outcome after complete resection." 01/01/2019 - "While steroids are the mainstay of treatment in IgG4-related disease, surgical resection may be curative in solitary lesions presenting with compressive symptoms." 09/01/2020 - "This raised the possibility of immunoglobulin G4-related disease (IgG4-RD), which was effectively treated with oral steroids. " 03/01/2017 - "Steroid treatment is effective for IgG4-RD, but the long-term outcome still remains unclear."
4.	Adrenal Cortex Hormones (Corticosteroids)IBA 04/01/2020 - "The relationship between both entities may have therapeutic impact because IgG4-RD of the head and neck has a high remission rate with corticosteroids and immunosuppressive therapy. " 03/01/2017 - "Corticosteroids are therapeutic, but surgical resection may be necessary for temporal bone IgG4-RD to improve long-term remission." 02/01/2017 - "Systemic corticosteroids are generally effective in inducing IgG4-RD remission; however, recurrent or refractory cases are common. " 12/01/2016 - "Identification of IgG4-RD may prevent unnecessary surgery because corticosteroids may be effective in these patients." 05/01/2014 - "Corticosteroid is effective for orbital and systemic involvement in IgG4-RD."
5.	Immunoglobulin G (IgG)IBA 12/01/2011 - "This case was diagnosed as lymphoplasmacytic type IPT which appeared to be highly homologous with IgG4-related disease due to high serum levels of IgG4, pituitary involvements and the observed efficacy of PSL." 10/01/2012 - "Immunohistochemical staining of IgG and IgG4 is very helpful for a definite diagnosis of IgG4-related disease." 01/01/2016 - "The accumulation of a greater number of reports on the pathological investigation of cases of possible IgG4-related disease may help to elucidate the exact role of IgG4 in IgG4-related disorders. " 08/01/2023 - "Using expert clinical judgment as the gold standard for diagnosis of IgG4-RD, the performance (sensitivity, specificity, area under the curve (AUC) of the 2019 ACR/EULAR criteria for IgG4-RD was evaluated. " 01/01/2023 - "After the patient was diagnosed with IgG4-RD, a series of treatments improved skin lesions, relieved other symptoms, and decreased serum IgG4 levels. "
6.	Immunosuppressive Agents (Immunosuppressants)IBA 09/30/2022 - "Alternate-day GC treatment is a feasible option for patients with IgG4-RD, without a significant increase in disease flares particularly when combined with immunosuppressants." 05/01/2015 - "Currently available experimental evidence classifies IgG4-RD as an immune-mediated disorder, providing support to the use of immunosuppressants and possibly even more specific therapies targeting the B-cell and plasma cell lineages." 09/01/2017 - "Glucocorticoids and immunosuppressive agents were effective for IgG4-RD. " 08/09/2022 - "Following the diagnosis of IgG4-RD with KD, glucocorticoids with immunosuppressants were initiated; there was a prompt improvement in the patient's condition. " 10/01/2023 - "We conducted the current systematic review and meta-analysis to evaluate the efficacy and safety of the combination of glucocorticoid and immunosuppressive agents (IM) compared to glucocorticoid (GC) monotherapy for the treatment of immunoglobulin G4-related disease (IgG4-RD). "
7.	CytokinesIBA 04/20/2018 - "In this sample of patients with aggressive IgG4-RD and renal involvement, treatment aimed at depleting B cells and decreasing antibody and cytokine production was associated with a substantial, persistent increase in eGFR, and a definite improvement in immunologic, radiologic and histological parameters." 01/01/2016 - "Data from several studies indicate that patients who have a T-helper type 2 (Th2-) dominant immune response, which leads to the hyperproduction of Th2 cytokines, then progress to IgG4-RD. " 01/01/2022 - "To characterize the clinical features of IgG4-related disease (IgG4-RD) and analyze the peripheral T lymphocyte subsets and cytokine levels. " 01/01/2021 - "Understanding the role of these cells and cytokines implicated in the pathogenesis of IgG4-RD will aid in developing strategies for future disease treatment and drug development. " 01/01/2021 - "Adaptive immune cells, including different types of T and B cells, and cytokines secreted by these cells play a vital role in the pathogenesis of IgG4-RD. "
8.	iguratimodIBA 02/01/2020 - "Key messages • Iguratimod was effective for relapsed or refractory IgG4-RD patients. " 02/01/2020 - "To evaluate the therapeutic efficacy and safety of iguratimod on patients with relapsed or refractory IgG4-related disease (IgG4-RD). " 02/01/2020 - "Efficacy and safety of iguratimod on patients with relapsed or refractory IgG4-related disease." 01/01/2023 - "In recent years, clinical studies have found that iguratimod is effective in the treatment of RA, SLE, IGG4-RD, Sjogren 's syndrome, ankylosing spondylitis, interstitial lung disease, and other autoimmune diseases and rheumatic diseases. " 08/01/2019 - "The purpose of this study is to evaluate the therapeutic efficacy and safety of iguratimod plus corticosteroid as bridge therapy in the treatment of mild immunoglobulin G4-related disease (IgG4-RD). "
9.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2021 - "Improved clinical outcomes of tocilizumab versus cyclophosphamide for IgG4-related disease: insights from a prospective IgG4-related disease registry." 01/01/2017 - "Efficacy of Cyclophosphamide treatment for immunoglobulin G4-related disease with addition of glucocorticoids." 10/15/2020 - "IgG4-related disease (IgG4-RD) with KS was finally diagnosed, and high dose prednisolone with intravenous cyclophosphamide was initiated and thereafter the patient demonstrated a prompt improvement. " 01/01/2020 - "Comparison of the Effects of Cyclophosphamide and Mycophenolate Mofetil Treatment Against Immunoglobulin G4-Related Disease: A Retrospective Cohort Study." 01/01/2017 - "Aim to evaluate the efficacy and safety of glucocorticoid monotherapy vs combination therapy of cyclophosphamide (CYC) for IgG4 related disease (IgG4-RD). "
10.	Prednisolone (Predate)FDA LinkGeneric 06/01/2014 - "Oral prednisolone was effective in the treatment of IgG4-related disease. " 07/01/2021 - "There was no evidence of relapsing IgG4-related disease during prednisolone tapering. " 08/06/2020 - "The patient was then diagnosed with IgG4-RD and successfully treated with prednisolone. " 10/01/2018 - "This case shows that the therapeutic response to prednisolone significantly differs between glomerular lesions and interstitial lesions of IgG4-related kidney disease." 07/01/2017 - "Prednisolone treatment remains the standard of care in patients with IgG4-RD."

Therapies and Procedures

1.	Therapeutics 01/01/2023 - "B-cell depletion therapy is an emerging curative strategy that can significantly alleviate clinical manifestations and achieve remission in patients with IgG4-related disease. " 11/01/2020 - "Complete resection of a conspicuous and solitary IgG4-RD lesion in the frontal convexity is simple and could provide a cure with less-aggressive adjuvant therapy." 01/01/2020 - "Treatment of IgG4-RD patients with GCs and IM was associated with higher remission rates and lower relapse rates, as well as comparable safety profiles compared with GC, IM and RTX induction therapy. " 09/01/2021 - "RTX therapy is an effective treatment for IgG4-RD that is refractory to GCs. " 01/01/2017 - "RTX is effective for both induction therapy and treatment of relapses in IgG4-RD, but relapses are frequent after B-cell reconstitution. "
2.	Remission Induction 10/01/2018 - "The aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction. " 10/01/2018 - "The therapeutic goals of remission induction were defined as fulfilling each of the following after the 6-month remission induction stage: (1) ≥ 50% decline in the IgG4-RD responder index (RI); (2) GC tapered to maintenance dose; and (3) no relapse during GC tapering. " 01/01/2020 - "Fourteen patients with IgG4-RD were treated with RTX as induction of remission therapy at the San Raffaele Scientific Institute in Milan, Italy. " 06/01/2016 - "The objectives of this study were to review our experience with rituximab as remission induction in IgG4-RD, to clarify the duration of efficacy and to identify predictors of flare following treatment. " 09/01/2021 - "Also, it underlines that the management of the disease is through glucocorticoids (GCs) as the first-line agent for remission induction in all patients with active, untreated IgG4-RD. "
3.	Aftercare (After-Treatment) 09/25/2023 - "The mean IgG4-RD RI scores of all patients at baseline (7.75 ± 2.92) and after treatment (2.00 ± 0.76) were highly significantly different (P < 0.001). " 07/13/2023 - "The percentages and absolute numbers of ABCs (gated as CD21-T-bet+CD11c+) in CD19+ B cells raised remarkably in untreated IgG4-RD patients than HC, and reduced significantly after treatment. " 06/19/2020 - "Increasing awareness of this condition may influence the management of these patients, once patients with IgG4-related disease should be monitored after treatment, due to the risk of recurrence or involvement of other organs." 05/01/2018 - "Serum sIL-2R levels decreased significantly after treatment in patients with IgG4-RD and pSS. " 01/01/2018 - "We studied the levels of sIL-2R in 26 histologically proven IgG4-RD patients with available serum sIL-2R and compared them to those in newly diagnosed and untreated sarcoidosis patients (n = 78) and controls (n = 101) and the serum sIL-2R levels in patients after treatment of IgG4-RD (n = 15). "
4.	Bridge Therapy 08/01/2019 - "The purpose of this study is to evaluate the therapeutic efficacy and safety of iguratimod plus corticosteroid as bridge therapy in the treatment of mild immunoglobulin G4-related disease (IgG4-RD). " 08/01/2019 - "Efficacy and safety of iguratimod plus corticosteroid as bridge therapy in treating mild IgG4-related diseases: A prospective clinical trial." 08/01/2019 - "Iguratimod plus corticosteroid as bridge therapy is effective for the treatment of mild IgG4-RD, it can improve the clinical symptoms, reduce serum IgG and IgG4 levels, especially plasmablasts/plasma cells and memory B cells. "
5.	Stents 05/01/2022 - "We report a case of supraglottic stenosis due to IgG4-RD together with a novel treatment strategy of employing a laryngeal stent. " 10/01/2021 - "This case highlights the importance for cardiologists to consider IgG4-RD as a rare but possible association with stent thrombosis." 10/01/2021 - "Recurrent Stent Thrombosis Secondary to Immunoglobulin G4-Related Disease." 10/01/2021 - "We report the first case of a patient with multiple myocardial infarctions caused by recurrent stent thrombosis associated with IgG4-RD, which resolved upon treatment of IgG4-RD. " 03/01/2019 - "Indication for treatment was DVT (n = 4), recurrent DVT (n = 1), stent thrombosis (n = 3), high-grade extrinsic narrowing of the inferior vena cava (IVC) due to immunoglobulin G4-related disease (n = 1), and IVC obstruction from liver tumor invasion (n = 1). "