Update on classification, diagnosis, and management of immunoglobulin G4-related disease

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized chronic fibro-inflammatory autoimmune disease, and its recognition has been constantly increasing worldwide over the last few years. A correct and timely recognition, as well as appropriate intervention, is crucial for the treatment of IgG4-RD. For certain subtypes of IgG4-RD, organ-specific criteria are formulated to make the diagnosis more accurate. New biomarkers have emerged in the recent years to aid the disease diagnosis, its prognosis prediction, as well as therapy response monitoring. Although recurrence is very common in IgG4-RD, glucocorticoid is still the first-line treatment for the majority of patients. The factors that affect the likelihood of disease relapse are multifaceted. The selection strategy of various steroid-sparing agents is still being explored. Besides, when patients have special sites involvement leading to severe clinical conditions, surgical operation or interventional therapy should also be considered. An update on classification, diagnosis, and management of IgG4-RD is provided in the current study to fully elucidate the recommended clinical practice of this mysterious disease.

Serial changes in anxiety levels related to corticosteroid use: A single-center prospective study

“…The doses of corticosteroids were determined based on the type of disease, disease activity, and body weight. [1,2,[5][6][7][8][9] Patients who had a history of corticosteroid therapy or had cognitive disorders were excluded. This study was approved by the Ethics Committee of Yamaguchi University Hospital (Control No.…”

Immunoglobulin G4-related disease: A narrative review

“…Since disease biomarkers might be normal at illness onset and are, therefore, useless for determining treatment response and relapse prediction, none of the currently available disease biomarkers alone can generally be considered a trustworthy mirror of disease activity [32]. However, the following actions were recommended to monitor disease activity: Clinical evaluation of all possibly affected organs; peripheral eosinophil count monitoring; measurement of total serum levels of IgE, IgG, and IgG4; and inflammatory markers, and tracking changes seen in the results of images of affected organs [33].…”

Comparison of clinical features and outcomes of proliferative, fibrotic, and mixed subtypes of IgG4-related disease: A retrospective cohort study

“…[ 8 , 9 ] The efficacy of glucocorticoids (GCs), the first-line agents for IgG4-RD, has been verified in numerous retrospective studies. [ 10 , 11 ] Immunosuppressants (IMs) are optionally used in combination with GCs to provide additional efficacy and steroid-sparing effects. [ 12 13 14 15 ] However, relapse frequently occurs following GCs tapering or discontinuation, reportedly occurring in more than 30% of IgG4-RD patients, [ 16 17 18 19 ] which makes the management of IgG4-RD challenging.…”