Beyond Glucocorticoids for IgG4-Related Disease

IgG4-related disease is an increasingly recognized fibroinflammatory condition with protean manifestations. The disorder is characterized by inflammatory mass lesions that can involve many different organs and organ systems — including the pancreas and biliary tract, orbital structures, salivary and lacrimal glands, lungs, retroperitoneum, and kidneys — and can also cause aortitis and periaortitis.1 The effects of the disease relate to resultant damage in the affected organ systems. It often can be a relapsing disorder, and the optimal approach to inducing or maintaining remission has not been well defined. The etiologic factors of IgG4-related disease are uncertain, but the histopathological characteristics . . .