Autoimmune pancreatitis

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Autoimmune pancreatitis
Synonyms	AIP
Pronounce	N/A
Specialty	N/A
Symptoms	Jaundice, abdominal pain, weight loss, diabetes mellitus
Complications	Pancreatic cancer, bile duct obstruction, diabetes mellitus
Onset	Typically in middle age
Duration	Chronic
Types	Type 1 (lymphoplasmacytic sclerosing pancreatitis), Type 2 (idiopathic duct-centric pancreatitis)
Causes	Autoimmune disease
Risks	IgG4-related disease, other autoimmune diseases
Diagnosis	Imaging studies, serology, biopsy
Differential diagnosis	Pancreatic cancer, chronic pancreatitis
Prevention	N/A
Treatment	Corticosteroids, immunosuppressive drugs
Medication	Prednisone, azathioprine
Prognosis	Generally good with treatment
Frequency	Rare
Deaths	N/A

A form of pancreatitis caused by the body's immune system attacking the pancreas

Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis characterized by an autoimmune inflammatory process that affects the pancreas. It is part of a spectrum of autoimmune diseases and is often associated with other systemic autoimmune conditions.

Classification

Autoimmune pancreatitis is classified into two types:

• Type 1 AIP: Also known as lymphoplasmacytic sclerosing pancreatitis, this type is associated with IgG4-related disease. It is characterized by elevated serum IgG4 levels and the presence of IgG4-positive plasma cells in the pancreas.
• Type 2 AIP: Also known as idiopathic duct-centric pancreatitis, this type is not associated with IgG4-related disease. It is characterized by granulocytic epithelial lesions and is often associated with inflammatory bowel disease, such as ulcerative colitis.

Signs and symptoms

Patients with autoimmune pancreatitis may present with a variety of symptoms, including:

• Jaundice due to bile duct obstruction
• Abdominal pain
• Weight loss
• Diabetes mellitus
• Pancreatic insufficiency leading to steatorrhea

Diagnosis

The diagnosis of autoimmune pancreatitis is based on a combination of clinical, serological, radiological, and histological findings. Key diagnostic criteria include:

• Elevated serum IgG4 levels (particularly in Type 1 AIP)
• Characteristic imaging findings on CT scan or MRI, such as diffuse or focal pancreatic enlargement and a "sausage-shaped" pancreas
• Histological examination showing lymphoplasmacytic infiltration and fibrosis
• Response to corticosteroid therapy

Treatment

The primary treatment for autoimmune pancreatitis is corticosteroid therapy, which typically leads to rapid improvement in symptoms and imaging findings. In some cases, additional immunosuppressive agents such as azathioprine or mycophenolate mofetil may be used to maintain remission.

Prognosis

With appropriate treatment, the prognosis for autoimmune pancreatitis is generally good. However, relapses can occur, and long-term follow-up is necessary to monitor for recurrence and manage any associated autoimmune conditions.

See also

• Pancreatitis
• Chronic pancreatitis
• IgG4-related disease
• Autoimmune disease