Thanks to John for presenting the case of a middle aged man from Vietnam with history of smoking who presented to the hospital with painless jaundice with imaging concerning for malignancy, found to have IgG4 related autoimmune pancreatitis!
- For imaging of the biliary tree:
- Ultrasound is best for stones
- CT is better for parenchymal disease
- ERCP/MRCP is best for intraductal masses or stones
- IgG4 related disease is more common in men >50 years of age. It can affect many organs, similar to sarcoid.
- It is an inflammatory and fibrotic systemic conditions where organs form tumefactive lesions rich in IgG4 plasma cells.
- Diagnosis requires biopsy. Serum IgG levels may be normal even in active disease. However, a significantly elevated level is highly sensitive/specific (>95%) for IgG4 related disease.
- Treatment involves steroids + immunomodulating therapy.
Approach to high bilis!
DDx for painless jaundice:
- Cancer (pancreatic, cholangiocarcinoma)
- Viral hepatitis
IgG4 related disease
- First described in 2003
- Majority men (62-83%) and > 50 years of age.
- Inflammatory and Fibrotic systemic condition where organs have tumefactive (tumor forming) lesions with an infiltrate rich in IgG4 plasma cells and often elevated IgG4 serum levels (but not always!)
- The pathophys is poorly understood but thought to be a combination of autoimmune and allergic mediated processes.
- Subacute onset, typically few systemic signs/symptoms
- 30% of those with autoimmune pancreatitis also have tubulointerstitial nephritis at presentation.
- Pancreatic involvement
- Manifestations include a uniformly enlarged pancreas (sausage pancreas on imaging), pancreatic mass which can mimic cancer, recurrent pancreatitis, or strictures.
- Biliary involvement
- Biliary strictures leading to obstructive jaundice as well as sclerosing cholangitis
- ANY organ can be affected (eg: thyroiditis, interstitial nephritis, salivary involvement), much like sarcoid
Spectrum of IgG4-related disease
- Need tissue biopsy for diagnosis. Serum serologies are only suggestive.
- Steroids + immunomodulating therapy.
Etiologies of Chronic Pancreatitis (progressive inflammatory changes in the pancreas that result in permanent structural damage and histologic fibrosis)
- Alcohol abuse(45%) as well as cigarette use
- Recurrent acute pancreatitis
- Genetic (eg: CFTR, SPINK mutations)
- Chronic ductal obstruction
- Systemic diseases (eg: SLE, hyperparathyroidism, hypertriglyceridemia)
- Autoimmune: Can be Type 1 (part of IgG4 related disease) or Type 2 (idiopathic)
Clinical manifestations of chronic pancreatitis
- Can be ASYMPTOMATIC
- Epigastric abdominal pain most common symptom however
- Pancreatic insufficiency (only after 90 %of pancreatic function lost) and manifests as steatorrhea and glucose intolerance/diabetes
- Remember that chronic pancreatitis puts you at increased risk for PANCREATIC CANCER
- Amylase/Lipase usually NORMAL so not as helpful
- 72 hour quantitative fecal fat (steatorrhea alone is non-specific!)
- Fecal elastasehas high sensitivity and specificity for chronic pancreatitis
- KUB can show calcificationshinting towards chronic pancreatitis and MRCP/ultrasound can show pancreatic duct obstructions, dilations, strictures or fluid collections
- Alcohol and smoking cessation!
- Creon supplementation, may also need fat-soluble (A,D,K,E) supplementation
- Analgesics for abdominal pain which is extremely hard to control. Minimize opioids but may be necessary for refractory pain.
- Specialized approaches include celiac nerve blocks, endoscopic surgery and surgical resection