title: Endoscopically proven case of rapid esophagogastric variceal progression and rupture as a result of portal hypertension with liver sarcoidosis.
abstract: Sarcoidosis is a multi-systemic disease of unknown etiology that results in the development of non-caseating epithelioid granulomas. The liver is the third most frequently involved organ after the lymph nodes and the lungs. Most cases of liver sarcoidosis do not present with symptoms and involve minimal liver dysfunction, but some cases display progression to portal hypertension and liver cirrhosis, and finally to liver failure. The mechanism and the risk of progression in liver sarcoidosis are still unknown because of the diagnostic difficulty associated with this condition, and because follow-up examinations can only be done in an invasive manner. Here, we present an informative case of liver sarcoidosis with rapid progression of esophagogastric varices. Four months prior to the definitive diagnosis, no signs of varices were observed on endoscopy, and developmentof esophagogastric varices, rapid progression, and eventual rupture occurred in a short period of time. A liver biopsy, carried out after endoscopic sclerotherapy, revealed that granulomas primarily affected the portal area without fibrotic and cirrhotic changes, which is considered a primary cause of portal hypertension and esophagogastric varices. Following the liver biopsy, the patient was given systemic steroids and is currently receiving outpatient care. Thus, we should consider the possibility that liver sarcoidosis, even in the absence of cirrhotic changes, can cause serious events such as esophagogastric variceal rupture following rapid progression as a result of portal hypertension.
authors.affiliation: Division of Gastroenterology, Tohoku University Hospital, Sendai, Japan.
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Ara
authors.forename: Katsunori
authors.initials: K
authors.lastname: Iijima
authors.forename: Junya
authors.initials: J
authors.lastname: Honda
authors.forename: Toshimitsu
authors.initials: T
authors.lastname: Iwabuchi
authors.forename: Waku
authors.initials: W
authors.lastname: Hatta
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Endo
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Koike
authors.forename: Osamu
authors.initials: O
authors.lastname: Kimura
authors.forename: Yasuteru
authors.initials: Y
authors.lastname: Kondo
authors.forename: Toyohiko
authors.initials: T
authors.lastname: Yuki
authors.forename: Tooru
authors.initials: T
authors.lastname: Shimosegawa
mesh_terms: D000328:Adult; D018450:Disease Progression; D016145:Endoscopy, Digestive System; D004932:Esophageal and Gastric Varices / Q000175:diagnosis* / Q000209:etiology*; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications*; D008107:Liver Diseases / Q000150:complications*; D012421:Rupture; D012507:Sarcoidosis / Q000150:complications*
keywords: esophageal varices; liver sarcoidosis; portal hypertension