Management of IgG4-related disease

Abstract

IgG4-related disease was unrecognised as a unified disease entity until this century, yet in a short period of time the disease has been appreciated to have a worldwide distribution, and its clinical, pathological, and radiological features have been described in considerable detail. The disease has strong organ predilections, and many of the clinical presentations of disease are increasingly familiar to both generalists and specialists. Early recognition of IgG4-related disease is crucial because although the disease is highly treatable, it can lead to serious organ damage and even death if undiagnosed until advanced stages. Its indolent nature often makes diagnosis challenging, and IgG4-related disease is one of the great mimickers of other diseases in the current era. Glucocorticoids are an effective treatment for IgG4-related disease, but their long-term use is problematic in a disease that frequently affects middle-aged to elderly individuals and often leads to pancreatic dysfunction. Our understanding of the pathophysiology of the disease is surprisingly advanced given the relatively recent recognition of this condition. Insights into disease pathophysiology offer the possibility of a variety of targeted treatment approaches. Looking ahead, biological therapies could profoundly alter the way in which IgG4-related disease is managed, permitting the use of specific therapies that are tailored to patients' clinical phenotypes.