+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Gastric variceal bleeding uncovering a rare association of CREST syndrome, primary biliary cirrhosis, nodular regenerative hyperplasia and pulmonary hypertension



Gastric variceal bleeding uncovering a rare association of CREST syndrome, primary biliary cirrhosis, nodular regenerative hyperplasia and pulmonary hypertension



European Journal of Gastroenterology and Hepatology 22(9): 1145-1148



A 73-year-old woman was admitted after the first upper gastric tract haemorrhage due to gastric variceal bleeding. A CREST syndrome associated with Hashimoto's thyroiditis, Gougerot-Sjögren syndrome, cryoglobulinaemia and complicated with severe pulmonary hypertension was diagnosed. Liver histology found precirrhotic lesions of primary biliary cirrhosis (PBC) and nodular regenerative hyperplasia (NRH). Collagen diseases are often associated with liver test abnormalities and liver disease usually associated with CREST syndrome is PBC. NRH has been found in association with collagen diseases but also with haematological diseases or drugs or with autoimmune diseases, such as PBC. This case shows the association of PBC and NRH with porto pulmonary hypertension in CREST syndrome.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 053353252

Download citation: RISBibTeXText

PMID: 20485183

DOI: 10.1097/meg.0b013e32833ab83a


Related references

A case of nodular regenerative hyperplasia of the liver, CREST syndrome, and primary biliary cirrhosis. Gut 31(11): 1335-1336, 1990

CREST syndrome: nodular regenerative hyperplasia of the liver and primary biliary cirrhosis an overlap syndrome?. Gut 31(4): 483-484, 1990

Nodular regenerative hyperplasia of the liver, CREST syndrome and primary biliary cirrhosis: an overlap syndrome?. Gut 30(10): 1430-1433, 1989

Nodular regenerative hyperplasia of the liver and primary biliary cirrhosis. A fortuitous association?. Gastroenterologie Clinique et Biologique 10(11): 764-766, 1986

Primary biliary cirrhosis, CRST syndrome, Sjögren's syndrome and nodular regenerative liver hyperplasia. Ceskoslovenska Gastroenterologie a Vyziva 36(8): 429-436, 1982

Esophageal variceal bleeding in feltys syndrome associated with nodular regenerative hyperplasia. Annals of the Rheumatic Diseases 37(2): 183-186, 1978

Oesophageal variceal bleeding in Felty's syndrome associated with nodular regenerative hyperplasia. Annals of the Rheumatic Diseases 37(2): 183-186, 1978

Nodular regenerative hyperplasia and primary biliary cirrhosis. Gastroenterologie Clinique et Biologique 15(11): 856-858, 1991

Nodular regenerative hepatic hyperplasia associated with primary biliary cirrhosis. Revista Clinica Espanola 191(8): 433-434, 1992

Nodular regenerative hyperplasia of the liver nrh associated with primary biliary cirrhosis pbc. Journal of Hepatology 13(Suppl. 2): S110, 1991

Primary biliary cirrhosis and CREST-syndrome, a classical but rare association. Clinical and Experimental Rheumatology 5(2): 190-192, 1987

A case of nodular regenerative hyperplasia of liver that mimicked primary biliary cirrhosis. Korean Journal of Hepatology 10(4): 313-318, 2004

Nodular regenerative hyperplasia of the liver in early histological stages of primary biliary cirrhosis. Gastroenterology 102(4 Pt 1): 1319-1324, 1992

Hepatocellular carcinoma arising in primary biliary cirrhosis presenting with nodular regenerative hyperplasia: report of an autopsy case. Journal of Gastroenterology and Hepatology 21(12): 1866-1868, 2006

Nodular regenerative hyperplasia of the liver associated with primary pulmonary hypertension. Human Pathology 19(6): 726-731, 1988