Advances in the diagnosis and management of IgG4 related disease

Abstract

IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multifaceted presentation, IgG4 related disease represents a great mimicker of many neoplastic, inflammatory, and infectious conditions. Histopathology remains key to diagnosis because reliable biomarkers are lacking. Recently released classification criteria will be invaluable in improving early recognition of the disease. IgG4 related disease is highly treatable and responds promptly to glucocorticoids, but it can lead to end stage organ failure and even death if unrecognized. Prolonged courses of corticosteroids are often needed to maintain remission because the disease relapses in most patients. Rapid advancement in our understanding of the pathophysiology of IgG4 related disease is leading to the identification of novel therapeutic targets and possible personalized approaches to treatment.

Dupilumab as a potential steroid-sparing treatment for IgG4-related disease

“…Although we strongly support the idea that, by interfering with IL-4 and IL-13 pathways, dupilumab might represent a promising biologic therapy for IgG4-RD, we would like to share with the authors our perplexities about the conclusions drawn from this case report 2…”

“…Current available treatments for IgG4-RD including glucocorticoids and B-cell depleting agents, in fact, are associated with many potential side effects, and their long-term use can become problematic in a disease that frequently affects middle-aged to elderly individuals 6–8. Given the central role of IL-4 and IL-13 in IgG4 class switch and tissue fibrosis, targeting these pathways has strong rationale in IgG4-RD 2. Whether dupilumab might find ideal application in disease phenotypes characterised by peripheral eosinophilia, elevation of serum IgG4 or IgE levels, or atopic manifestations remains speculative and deserves confirmation in randomised clinical trials 9…”

IgG4-related disease: the state of art

“…В развитии IgG4-ассоциированного заболения можно выделить две фазы -воспаление и фиброз [18,19]. В воспалительную фазу происходит клональная экспансия патогенных В-и Т-клеток, которые накапливаются в тканях и секретируют различные цитокины, хемокины, факторы роста и ферменты, поддерживающие воспаление и способствующие развитию фиброза [20,21].…”

“…Сывороточная концентрация IgG4 повышается у 55-97% больных с IgG4-ассоциированным заболеванием и коррелирует с числом пораженных органов [18]. M. Hao и соавт.…”

“…При использовании в качестве "отрезного" значения более высокого содержания IgG4 (2,70-2,80 г/л) специфичность увеличилась до 94,8% (95% ДИ 94,1-95,4%), однако чувствительность снизилась до 63,0% (95% ДИ 60,0-66,0%). Сывороточное содержание IgG4 может быть использовано в качестве скринингового показателя, однако необходимо учитывать возможность его повышения при других аутоиммунных заболеваниях, а также инфекциях и опухолях [18]. M. Ebbo и соавт.…”