Health Dictionary

Overview

Primary sclerosing cholangitis must be differentiated from causes of secondary sclerosing cholangitis.

Differentiating Primary sclerosing cholangitis from other Diseases

Primary sclerosing cholangitis must be differentiated from causes of secondary sclerosing cholangitis. These include:^[1]^[2]

Primary sclerosing cholangitis must be differentiated from other causes of jaundice and pruritis:

Classification of jaundice based on etiology	Disease	History and clinical manifestations				Diagnosis
		History and clinical manifestations				Lab Findings						Other blood tests	Other diagnostic
		Family history	Fever	RUQ Pain	Pruritis	AST	ALT	ALK	BLR Indirect	BLR Direct	Viral serology	Other blood tests	Other diagnostic
Cholestatic Jaundice	Primary sclerosing cholangitis	-/+	+	+	+	N/↑	N/↑	↑	N	↑	–	Beading on MRCP	Liver biopsy
	Common bile duct stone	-/+	-/+	+	+	N	N	↑	N	↑	–	Dilated ducts on sono	CT/ERCP
	Hepatitis A cholestatic type	–	-/+	+	+	N	N	↑	N	↑	+	HAV- AB	Abdominal ultrasound
	EBV / CMV hepatitis	–	-/+	+	+	N	N	↑	N	↑	+	Positive serology
	Primary biliary cirrhosis	-/+	–	-/+	+	N/↑	N/↑	↑	N	↑	–	AMA positive	Liver biopsy
	Pancreatic carcinoma	+	–	-/+	–	N/↑	N/↑	↑	N	↑	–	Mass on ultrasond	CT scan for diagnosis

Abbreviations: RUQ= Right upper quadrant of the abdomen, LUQ= Left upper quadrant, LLQ= Left lower quadrant, RLQ= Right lower quadrant, LFT= Liver function test, SIRS= Systemic inflammatory response syndrome, ERCP= Endoscopic retrograde cholangiopancreatography, IV= Intravenous, N= Normal, AMA= Anti mitochondrial antibodies, LDH= Lactate dehydrogenase, GI= Gastrointestinal, CXR= Chest X ray, IgA= Immunoglobulin A, IgG= Immunoglobulin G, IgM= Immunoglobulin M, CT= Computed tomography, PMN= Polymorphonuclear cells, ESR= Erythrocyte sedimentation rate, CRP= C-reactive protein, TS= Transferrin saturation, SF= Serum Ferritin, SMA= Superior mesenteric artery, SMV= Superior mesenteric vein, ECG= Electrocardiogram

Primary sclerosing cholangitis must be differentiated from other abdominal pain causes:

	Abdominal Pain	Fever	Rigors and chills	Nausea or vomiting	Jaundice	Diarrhea	Weight loss	Hypo- tension	Guarding	Rebound Tenderness	Lab Findings	Imaging
Disease	Clinical manifestations										Diagnosis		Comments
	Symptoms							Signs			Diagnosis
Primary sclerosing cholangitis	RUQ	+	−	−	+	−	−	−	−	−	Increased liver enzymes Increased IgM, IgG4 Anti-neutrophil cytoplasmic antibody (p-ANCA) Anti-nuclear antibody (ANA) Anti-smooth muscle antibody (Anti-Sm) Anti-endothelial antibody Anti-cardiolipin antibody	ERCP and MRCP shows Multiple segmental strictures Mural irregularities Biliary dilatation and diverticula Distortion of biliary tree	The risk of cholangiocarcinoma in patients with primary sclerosing cholangitis is 400 times higher than the risk in the general population.
Acute suppurative cholangitis	RUQ	+	+	+	+	−	−	+	+	+	Abnormal LFT WBC >10,000	Ultrasound shows biliary dilatation/stents/tumor	Septic shock occurs with features of SIRS
Acute cholangitis	RUQ	+	−	−	+	−	−	−	−	−	Abnormal LFT	Ultrasound shows biliary dilatation/stents/tumor	Biliary drainage (ERCP) + IV antibiotics
Acute cholecystitis	RUQ	+	−	+	+	−	−	−	−	−	Hyperbilirubinemia Leukocytosis	Ultrasound shows: Gallstone Inflammation	Murphy’s sign
Disease	Abdominal Pain	Fever	Rigors and chills	Nausea or vomiting	Jaundice	Diarrhea	Weight loss	Hypo- tension	Guarding	Rebound Tenderness	Lab Findings	Imaging	Comments
Acute pancreatitis	Epigastric	+	−	+	±	−	−	±	−	−	Increased amylase / lipase	Ultrasound shows evidence of inflammation CT scan shows severity of pancreatitis	Pain radiation to back
Chronic pancreatitis	Epigastric	−	−	±	±	+	+	−	−	−	Increased amylase / lipase Increased stool fat content Pancreatic function test	CT scan Calcification Pseudocyst Dilation of main pancreatic duct	Predisposes to pancreatic cancer
Pancreatic carcinoma	Epigastric	−	−	+	+	+	+	−	−	−	↑ Alkaline phosphatase ↑ serum bilirubin ↑ gamma-glutamyl transpeptidase ↑ CA 19-9	MDCT with PET/CT MRI	Skin manifestations may include: Bullous pemphigoid Cicatricial pemphigoid Migratory superficial thrombophlebitis (classic Trousseau’s syndrome)
Primary biliary cirrhosis	RUQ/Epigastric	−	−	−	+	−	−	−	−	−	Increased AMA level, abnormal LFTs	ERCP	Pruritis
Cholelithiasis	RUQ/Epigastric	±	−	±	±	−	−	−	−	−	Leukocytosis	Ultrasound shows gallstone	Fatty food intolerance
Biliary colic	RUQ	−	−	+	+	−	−	−	−	−	↑ bilirubin and alkaline phosphatase	Ultrasound
Disease	Abdominal Pain	Fever	Rigors and chills	Nausea or vomiting	Jaundice	Diarrhea	Weight loss	Hypo- tension	Guarding	Rebound Tenderness	Lab Findings	Imaging	Comments
Inflammatory bowel disease	Diffuse	±	−	−	±	+	+	−	−	−	Anti-neutrophil cytoplasmic antibody (P-ANCA) in Ulcerative colitis Anti saccharomyces cerevisiae antibodies (ASCA) in Crohn’s disease	String sign on abdominal x-ray in Crohn’s disease	Extra intestinal findings: Uveitis Arthritis
Whipple’s disease	Diffuse	±	−	−	±	+	+	±	−	−	Thrombocytopenia Hypoalbuminemia Small intestinal biopsy for Tropheryma whipplei	Endoscopy is used to confirm diagnosis. Images used to find complications Chest and joint x-ray CT MRI Echocardiography	Extra intestinal findings: Uveitis Endocarditis Encephalitis Dementia Hepatosplenomegaly Arthritis Ascites
Viral hepatitis	RUQ	+	−	+	+	Positive in Hep A and E	+	Positive in fulminant hepatitis	Positive in acute	+	Abnormal LFTs Viral serology	US	Hep A and E have fecal-oral route of transmission Hep B and C transmits via blood transfusion and sexual contact.
Disease	Abdominal Pain	Fever	Rigors and chills	Nausea or vomiting	Jaundice	Diarrhea	Weight loss	Hypo- tension	Guarding	Rebound Tenderness	Lab Findings	Imaging	Comments
Liver abscess	RUQ	+	+	+	+	±	+	+	+	±	CBC Blood cultures Abnormal liver function tests	US CT
Hepatocellular carcinoma/Metastasis	RUQ	+	−	−	+	−	+	−	−	−	High levels of AFP in serum Abnormal liver function tests	US CT Liver biopsy	Other symptoms: Splenomegaly Variceal bleeding Ascites Spider nevi Asterixis
Cirrhosis	RUQ	−	−	−	+	−	+	+	−	−	Hypoalbuminemia Prolonged PT Abnormal LFTs Hyponatremia Thrombocytopenia	US Nodular, shrunken liver Ascites	Stigmata of liver disease Cruveilhier- Baumgarten murmur
Spontaneous bacterial peritonitis	Diffuse	+	−	−	Positive in cirrhotic patients	+	−	±	+	+	Ascitic fluid PMN>250 cells/mm³ Culture: Positive for single organism	Ultrasound for evaluation of liver cirrhosis

References

↑ Lazaridis KN, LaRusso NF (2016). “Primary Sclerosing Cholangitis”. N. Engl. J. Med. 375 (12): 1161–70. doi:10.1056/NEJMra1506330. PMID 27653566.
↑ Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ (2010). “Diagnosis and management of primary sclerosing cholangitis”. Hepatology. 51 (2): 660–78. doi:10.1002/hep.23294. PMID 20101749.

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[pmid27653566-1] Lazaridis KN, LaRusso NF (2016). “Primary Sclerosing Cholangitis”. N. Engl. J. Med. 375 (12): 1161–70. doi:10.1056/NEJMra1506330. PMID 27653566.

[pmid20101749-2] Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ (2010). “Diagnosis and management of primary sclerosing cholangitis”. Hepatology. 51 (2): 660–78. doi:10.1002/hep.23294. PMID 20101749.

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Health Dictionary

Primary sclerosing cholangitis differential diagnosis

Introduction

Overview

Differentiating Primary sclerosing cholangitis from other Diseases

References