Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees.
Diagnostic Criteria of Primary Sclerosing Cholangitis (PSC)
It is necessary to exclude IgG4-related sclerosing cholangitis, secondary sclerosing cholangitis caused by diseases with an obvious pathogenesis, and malignant diseases such as biliary cancer.
A. Diagnostic items
I. Major items
A. Biliary tract imaging
1) Cholangiographic findings characteristic of PSC
2) Cholangiographic findings not specific to PSC
B. An increased alkaline phosphatase level
II. Minor items
a. Association with inflammatory bowel disease
b. Liver histology: Fibrous cholangitis/onion skin lesion
B. Diagnosis
Definite diagnosis
- A1) +B
- A1) +a
- A1) +b
- A2) +B+a+b
Probable diagnosis
- A1)
- A2) +B+a
- A2) +B+b
- A2) +a+b
Possible diagnosis
- A2) +a
- A2) +b
Lists of secondary sclerosing cholangitis
Congenital
- Caroli’s disease
- Cystic fibrosis
Chronic obstruction
- Choledocholithiasis
- Biliary stricutures (secondary to surgical trauma, chronic pancreatitis)
- Anastomotic stricutures in liver graft
- Neoplasmas (benign, malignant, metastatic)
Infectious
- Bacterial cholangitis
- Recurrent pyogenic cholangitis
- Parastic infection (cryptosporidiosis, microsporidiosis)
- Cytomegalovirus infection
Toxic
- Accidental alcohol, formaldehyde, hypertonic saline instillation in the bile ducts
Immunologic
- Eosinophilic cholangitis
- Acquired immunodeficiency
Ischemic
- Vascular trauma
- Posttraumatic sclerosing cholangitis
- Post-transplantation hepatic artery thrombosis
- Hepatic allograft rejection (acute, chronic)
- Intra-arterial, chemotherapy-related injury
- Transcatheter arterial embolization therapy
Infiltrative disorders
- Systemic vasculitis
- Amyloidosis
- Radiation injury
- Sarcoidosis
- Systemic mastocytosis
- Hypereosinophilic syndrome
- Hodgkin’s disease
References:
- Nakazawa T, Notohara K, Tazuma S, Tanaka A, Isayama H, Tsuyuguchi T, Mori T, Takikawa H. The 2016 diagnostic criteria for primary sclerosing cholangitis. J Gastroenterol. 2016 Dec 5. [Medline]
- Lazaridis KN, LaRusso NF. Primary Sclerosing Cholangitis. N Engl J Med. 2016 Sep 22;375(12):1161-70. [Medline]
- Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T. Diagnosis of IgG4-related sclerosing cholangitis. World J Gastroenterol. 2013 Nov 21;19(43):7661-70. [Medline]
Created Jan 12, 2017.
