Autoimmune pancreatitis articles from across Nature Portfolio

Autoimmune pancreatitis and IgG4-related cholangitis are frequent manifestations of IgG4-related disease (IgG4-RD). Here, Löhr and colleagues present current understanding of the pathophysiology of IgG4-RD and describe all IgG4-RD manifestations in the digestive tract. Diagnosis, prognosis and treatment of IgG4-RD are also discussed.

IgG4-related hepatobiliary diseases are part of a multiorgan fibroinflammatory condition termed IgG4-related disease. In this Review, Chapman and Culver provide an overview on the natural history, manifestations and aetiopathogenesis of IgG4-related hepatobiliary disease with an emphasis on IgG4-sclerosing cholangitis.

IgG4-related disease can affect any organ system, including the kidney. Renal involvement is an indication for prompt treatment, owing to an increased risk of chronic kidney disease. In this Review, Frank Cortazar and John Stone provide an overview of systemic IgG4-related disease before describing in detail the renal manifestations of IgG4-related tubulointerstitial nephritis and membranous glomerulonephropathy secondary to IgG4-related disease. They consider the pathophysiology, as well as current and future treatment options.

Autoimmune pancreatitis (AIP) is becoming increasingly recognized as a unique form of chronic pancreatitis. Its diagnosis, based on clinical and pathological parameters, is challenging. AIP is currently divided clinicopathologically into type 1 and type 2. Histological diagnostic criteria for type 1 and type 2 AIP have now been proposed by an international consensus study.