<unk>  may cause a variety of symptoms and signs, which include pancreatic and biliary (bile duct) manifestations, as well as systemic effects of the disease. Two-thirds of patients present with either painless jaundice due to bile duct obstruction or a "mass" in the head of the pancreas, mimicking carcinoma. As such, a thorough evaluation to rule out cancer is important in cases of suspected<unk> . Type 1<unk>  typically presents in a 60–70 year old male with painless jaundice. In some cases, imaging reveals a mass in the pancreas or diffuse pancreatic enlargement. Narrowing in the pancreatic duct called strictures may occur. Rarely, Type 1<unk>  presents with acute pancreatitis. Type 1<unk>  presents with manifestations of<unk>  disease (IgG4 related) in at least half of cases. The most common form of systemic involvement is cholangitis, which occurs in up to 80 percent of cases of Type 1<unk> . Additional manifestations include inflammation in the salivary glands (Sjögren syndrome), in the lungs resulting in scarring (pulmonary fibrosis) and nodules, scarring within the chest cavity (mediastinal fibrosis) or in the anatomic space behind the abdomen (retroperitoneal fibrosis) and inflammation in the kidneys (tubulointerstitial nephritis).<unk>  is characterized by the following features: # Scleral Icterus (yellow eyes), jaundice (yellow skin) which is usually painless, usually without acute attacks of pancreatitis. # Relatively mild symptoms, such as minimal weight loss or nausea. # Increased serum levels of gamma globulins, immunoglobulin G (IgG) or IgG4. # The presence of serum autoantibodies such as anti-nuclear antibody (ANA), anti-lactoferrin antibody, anti-carbonic anhydrase II antibody, and rheumatoid factor (RF). # Contrast-enhanced CT demonstrates a diffusely enlarged (sausage-shaped) pancreas. # Diffuse irregular narrowing of the main pancreatic duct, and stenosis of the intrapancreatic bile duct on endoscopic retrograde cholangiopancreatography (ERCP). # Rare pancreatic calcification or cyst formation. # Marked responsiveness to treatment with corticosteroids.
Autoimmune pancreatitis
Pancreatic cancer
Hereditary pancreatitis
Fibrocalculous pancreatopathy
Chronic pancreatitis
Biliary pseudolithiasis
Biliary fistula
Sialolithiasis