A case of idiopathic portal hypertension after renal transplantation

Abstract

A case of idiopathic portal hypertension (IPH) developing after renal transplantation is reported. A 33-year-old Japanese male who had undergone renal transplantation 8 years previously was transferred to our hospital because of hematemesis from ruptured esophageal varices. He had no history of any liver disease before the renal transplantation, but had a history of receiving blood transfusion. Abdominal computed tomography (CT) and ultrasonography revealed marked splenomegaly and collateral channels, but no obliteration which might cause portal hypertension in the hepatic or portal vein. No findings suggestive of hepatitis or liver cirrhosis were found either macroscopically on laparoscopy or by liver biopsy. Light microscopic study of the liver biopsy specimen showed mild periportal fibrosis, inconspicuous portal branches in the most peripheral tracts, but no pseudolobule formation or piecemeal necrosis. However collagen deposition was found in the perisinusoidal space and partly in intercellular space on electron microscopy. We consider that the development of portal hypertension in this case is responsible for the collagen deposition, which may be related to the administration of azathioprine after renal transplantation. There are few reports on IPH after renal transplantation, and it is stressed that a lower amount of azathioprine than previously reported may induce IPH under such conditions.

“Endemic” idiopathic portal hypertension: Report on 32 patients with non‐cirrhotic portal fibrosis

“…Some of the sporadic cases reported from North America and Western Europe have been due to chronic exposure to arsenics or vinyl chloride [2,[14][15][16]. This disease has also been reported following chemotherapy for cancer [17] and renal transplantation [18,19]. Association of IPH with autoimmune diseases has also been observed [19][20][21][22].…”

“…Sporadic cases have been documented to be related to chronic exposure to arsenics, vinyl chloride, or copper sulfate [14,15,16]. The disease has been observed following chemotherapy for cancer [17] and renal transplantation [18,19]. Several cases in association with autoimmune diseases have been reported worldwide [20][21][22].…”

Pediatrics

“…in 1979 after two adult patients presented with esophageal varices 1 and 5 years after renal transplantation. Following this case series, more reports of IPH in recipients of renal transplant while on azathioprine have been documented . All presented with complications of portal hypertension months to years after initiating therapy.…”

“…All presented with complications of portal hypertension months to years after initiating therapy. Perisinusoidal collagen deposition is characteristic in IPH after renal transplantation, but it is unclear to whether these changes are the cause or consequence of the disease process . Azathioprine therapy should be ceased once IPH has been diagnosed; however, there may be failure of resolution, or even progression of the disease, following cessation .…”

“…Perisinusoidal collagen deposition is characteristic in IPH after renal transplantation, but it is unclear to whether these changes are the cause or consequence of the disease process . Azathioprine therapy should be ceased once IPH has been diagnosed; however, there may be failure of resolution, or even progression of the disease, following cessation . Two case reports of patients with IPH after renal transplantation reported significant improvements in portal hypertension following splenectomy .…”