scholarly journals Clinical Variants of Primary Sclerosing Cholangitis: When Does Liver Biopsy Make the Diagnosis?

Author(s):  
Annarosa Floreani
2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S125-S125
Author(s):  
A Wilhelm ◽  
H L Stevenson ◽  
K Kline

Abstract Introduction/Objective Classic primary sclerosing cholangitis (PSC) involves extrahepatic and/or intrahepatic biliary ducts with segmental biliary strictures and dilatations that often allow the diagnosis to be made via cholangiogram. Small duct PSC (sdPSC) is a rare subtype that presents similarly with a cholestatic pattern of injury, yet due to the small size of involved ducts, a cholangiogram is non-diagnostic and diagnosis is dependent on clinical suspicion and liver biopsy. The histopathological features of sdPSC are often subtle and may easily be overlooked. Diagnosis of this entity- though difficult- is important, as early recognition can facilitate the identification of associated disease processes and life-threatening complications. Methods/Case Report We encountered a 33-year-old female presenting with intermittent pruritis, episodes of jaundice, and persistently elevated alkaline phosphatase who was misdiagnosed with only fatty liver at an outside institution. Evaluation with MRCP showed no abnormalities within the biliary tract and a liver biopsy was performed to aid in the diagnosis. The H&E and trichrome findings of atrophic bile ducts and some peribiliary sclerosis were extremely subtle and may have been overlooked without clinical suspicion. Cytokeratin 7 (CK7) highlighted cholangiolar metaplasia in hepatocytes and the bile ductular reaction that occurs in cholestatic disease states. A Rhodamine copper stain showed periportal deposition suggestive of chronic biliary obstruction. Use of CK7 and copper stains supported the presence of chronic biliary injury and suboptimal bile flow, confirming the diagnosis of sdPSC. Results (if a Case Study enter NA) NA Conclusion Diagnosis of sdPSC has historically relied on H&E and trichrome stains. In this case, the findings on H&E and trichrome stains were non-diagnostic, while the use of CK7 and copper stains confirmed the diagnosis of sdPSC. We recommend using CK7 and copper stains to evaluate for sdPSC.


2018 ◽  
Vol 154 (6) ◽  
pp. S-20 ◽  
Author(s):  
Céline Miard ◽  
Veronique Desfourneaux ◽  
Pauline Houssel-Debry ◽  
Yann Harnoy ◽  
Siproudhis Laurent ◽  
...  

2014 ◽  
Vol 05 (04) ◽  
pp. 168-170
Author(s):  
Nandeesh H. P. ◽  
Jeevan H. R. ◽  
Deepak Suvarna ◽  
Chandra Babu D. ◽  
Indrajit Suresh ◽  
...  

AbstractSclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.


2021 ◽  
Vol 8 (1) ◽  
pp. 24-28
Author(s):  
Mamun Al Mahtab ◽  
Sheikh M Noor-e-Alam ◽  
Dulal C Das ◽  
Merina Rahman ◽  
Abdullah A Mukit ◽  
...  

2009 ◽  
Vol 22 (10) ◽  
pp. 1287-1295 ◽  
Author(s):  
Vikram Deshpande ◽  
Nisha I Sainani ◽  
Raymond T Chung ◽  
Daniel S Pratt ◽  
Gilles Mentha ◽  
...  

1995 ◽  
Vol 48 (10) ◽  
pp. 933-935 ◽  
Author(s):  
R Olsson ◽  
I Hagerstrand ◽  
U Broome ◽  
A Danielsson ◽  
G Jarnerot ◽  
...  

2020 ◽  
Vol 7 (3) ◽  
pp. 5
Author(s):  
Maheep Singh Sangha ◽  
Aakash Aggarwal ◽  
Japmehr Sandhu

Primary sclerosing cholangitis (PSC) is a rare autoimmune disorder of the biliary system. PSC is fulminant in its course and leads to jaundice and biliary cirrhosis with specific radiological findings of the beading of larger ducts and pruning of smaller ducts. Though cholangiogram is the gold standard for PSC diagnosis, it may be inconclusive in patients with an early disease or small duct PSC or overlap with autoimmune hepatitis. These conditions may require a liver biopsy for confirmation of the underlying pathology. In this case report, we discuss one of these rare instances when a 24-year-old male was admitted with jaundice. During hospitalization, extensive workup, including cholangiograms, remained inconclusive, but based on a high suspicion of PSC, the patient underwent a liver biopsy, which confirmed our diagnoses


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