Service d'Hépato-Gastroentérologie, Hôpital du Haut-Lévêque, 33604 Pessac cedex, France.
Eur J Gastroenterol Hepatol. 2010 Sep;22(9):1145-8. doi: 10.1097/MEG.0b013e32833ab83a.
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.
一位 73 岁女性因胃静脉曲张出血首次出现上消化道出血后入院。诊断为与桥本甲状腺炎、干燥综合征、冷球蛋白血症相关的 CREST 综合征，并伴有严重肺动脉高压。肝脏组织学发现原发性胆汁性肝硬化 (PBC) 和结节性再生性增生 (NRH) 的早期肝硬化病变。胶原病常伴有肝功能异常，而 CREST 综合征相关的肝病通常为 PBC。NRH 已发现与胶原病有关，但也与血液疾病或药物或自身免疫性疾病有关，如 PBC。本病例显示 CREST 综合征中 PBC 和 NRH 与门肺高压相关。
