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Related Concept Videos

Gastritis-II: Pathophysiology01:17

Gastritis-II: Pathophysiology

Gastritis is marked by disruption of the mucosal barrier that usually protects the stomach tissue from digestive juices and manifests in acute and chronic forms.
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...
Chronic Pancreatitis I: Introduction01:25

Chronic Pancreatitis I: Introduction

Chronic pancreatitis is a long-standing, relapsing inflammation of the pancreas, characterized by irreversible damage to the gland. It results in progressive destruction of the pancreatic parenchyma, fibrosis, and eventual loss of both exocrine and endocrine function. The disease may evolve gradually after multiple episodes of acute pancreatitis or develop independently.EtiologyChronic pancreatitis can arise from a variety of causes:Alcohol use is the leading cause, accounting for 70–80% of...
Chronic Pancreatitis I: Introduction01:24

Chronic Pancreatitis I: Introduction

The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
Chronic Pancreatitis II: Pathophysiology01:21

Chronic Pancreatitis II: Pathophysiology

Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...
Acute Pancreatitis I: Introduction01:25

Acute Pancreatitis I: Introduction

Acute pancreatitis is the sudden inflammation of the pancreas caused by the early activation of digestive enzymes, leading to the autodigestion of pancreatic tissue. This results in local inflammation and, in severe cases, systemic complications.EtiologyUnderstanding the underlying causes is crucial, as identifying the etiology guides treatment and anticipates complications. Acute pancreatitis can be triggered by various factors, typically grouped into the following clinical categories.Biliary...
Acute Pancreatitis I: Introduction01:27

Acute Pancreatitis I: Introduction

Pancreatitis is inflammation of the pancreas, an organ located behind the stomach. It can be either acute or chronic.
Acute pancreatitis is characterized by rapid inflammation of the pancreas, often caused by factors like gallstone blockage or excessive alcohol consumption. Chronic pancreatitis, on the other hand, is a slow, progressive inflammation that may result from long-term alcohol abuse, obstructions in the pancreatic duct, or genetic factors.
The causes of acute pancreatitis include:

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Serum IgG4-negative autoimmune pancreatitis.

Terumi Kamisawa1, Kensuke Takuma, Taku Tabata

  • 1Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan. kamisawa@cick.jp

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Autoimmune pancreatitis (AIP) without elevated IgG4 levels presents distinct clinical and pathological features. These cases may represent distinct pancreatic conditions requiring further investigation.

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Area of Science:

  • Gastroenterology
  • Immunology
  • Pathology

Background:

  • Autoimmune pancreatitis (AIP) is often associated with IgG4-related disease.
  • However, approximately 20% of AIP patients lack elevated serum IgG4 levels.
  • The pathophysiology of these IgG4-negative AIP cases remains unclear.

Purpose of the Study:

  • To investigate the distinct clinicopathological features of AIP patients without elevated serum IgG4 levels.
  • To compare these features with those of AIP patients who do have elevated IgG4 levels.

Main Methods:

  • Fifty-eight AIP patients were classified into IgG4-positive (serum IgG4 >135 mg/dl) and IgG4-negative groups.
  • Clinical, serological, radiological, and histological findings were compared between groups.
  • Salivary and lacrimal gland function was also assessed.

Main Results:

  • IgG4-negative AIP patients showed a higher female ratio, less obstructive jaundice, and more abdominal pain/acute pancreatitis.
  • Pancreatic body/tail swelling was more common in IgG4-negative AIP.
  • Extrapancreatic lesions, salivary/lacrimal dysfunction, and IgG4-positive plasma cell infiltration in the gastric mucosa were less frequent.

Conclusions:

  • Clinicopathological characteristics of IgG4-negative AIP differ significantly from IgG4-positive AIP.
  • Some IgG4-negative AIP cases exhibit pancreatic-confined lymphoplasmacytic sclerosing pancreatitis (LPSP).
  • Further research is needed to determine if IgG4-negative AIP encompasses idiopathic duct-centric pancreatitis or other forms of sclerosing pancreatitis.