Pancreatic cancer and autoimmune diseases: An association sustained by computational and epidemiological case-control approaches

Miroslav Vujasinovic | Maalis 2020 | Onkologia ja Hematologia |

Miroslav Vujasinovic
MD, PhD,
senior attending physician,
Gastrocentrum,
Karolinska University Hospital,
Stockholm, Sweden

J.-Matthias Löhr
Professor, MD,
Karolinska Institutet,
CLINTEC, and Gastrocentrum,
Karolinska University Hospital,
Stockholm, Sweden

Autoimmune pancreatitis (AIP) is considered a pancreatic manifestation of a newly proposed disease condition, IgG4-related disease (IgG4-RD). Recent epidemiological studies have addressed the incidence of cancer in patients with AIP and/or IgG4-RD with conflicting results. In this review, we discuss an association with cancer in patients with type 1 AIP and IgG4-RD. Autoimmune pancreatitis (AIP) is a special form of chronic pancreatitis with strong lymphocytic infiltration as the pathological hallmark and two distinct histopathological subtypes: lymphoplasmacytic sclerosing pancreatitis (LPSP, AIP type 1) and idiopathic duct-centric pancreatitis (IDCP, AIP type 2).1 The disease was first described by Sarles in 19612 who described chronic inflammatory sclerosis of the pancreas; it was definitely named autoimmune pancreatitis in 1995 by Yoshida and colleagues when clinicopathological similarities to autoimmune hepatitis were noticed.3 Over the last two decades, many diagnostic criteria for AIP have been proposed in the literature.4-9 According to the International Consensus Diagnostic Criteria (ICDC 2010), the diagnosis of AIP is based on the presence of one or more of the following factors: pancreatic parenchyma and pancreatic duct imaging, serum IgG4 level, other organ involvement, histology of the pancreas and response to steroid treatment.10 IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue-destructive lesions and organ failure.11 Over the last two decades, the disease has become recognised as a unified systemic disorder that links many individual organ conditions once considered to be unrelated. Involvement of nearly every anatomic site has been reported, but the most commonly affected organs or anatomic sites are the pancreas (autoimmune pancreatitis type 1), biliary tract, major salivary glands (submandibular, parotid), lacrimal glands, retroperitoneum, and lymph nodes.12 It is well established that long-standing chronic inflammation plays a critical role in the development of cancer through the process of inflammation-associated carcinogenesis.13 Cancer...