A case of idiopathic portal hypertension associated with rheumatoid arthritis

Sasajima, Tomomi; Suzuki, Tomohiro; Mori, Kenji; Ichii, Osamu; Tai, Mayumi; Ochiai, Hironobu; Ejiri, Yutaka; Watanabe, Hiroshi; Ohira, Hiromasa; Obara, Katsutoshi; Satô, Yukio

doi:10.3109/s10165-006-0456-8

Cited by 9 publications

(4 citation statements)

References 14 publications

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“…Immune dysregulation is commonly associated with NCPH, with autoantibodies sometimes detectable in the serum . Autoimmune disorders such as systemic sclerosis, systemic lupus erythematosus (SLE), celiac disease, rheumatoid arthritis, Sjögren's syndrome and antiphospholipid antibody syndrome have been reported in association with NCPH; the first case of NRH was reported in association with Felty's syndrome in 1953. HIV has recently been implicated in the pathogenesis of NCPH.…”

Section: Discussionmentioning

confidence: 99%

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Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work

Agrawal

Rahmani

Nakkala

et al. 2013

Hepatology Research

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Nodular regenerative hyperplasia (NRH) and hepatoportal sclerosis, also known as obliterative portal venopathy (OPV), are two causes of non-cirrhotic portal hypertension (NCPH). NCPH is an increasingly recognized entity that can be seen in association with collagen vascular diseases and with the use of medications such as azathioprine and didanosine, but oftentimes the etiology remains unidentified. We herein report a case of NCPH occurring due to OPV and NRH in a 64-year-old woman with myasthenia gravis (MG), status post-thymectomy. Portal hypertension was diagnosed incidentally on computed tomography in the absence of predisposing factors. Extensive work-up to determine the etiology of any underlying liver disease was unrevealing. NRH and OPV were identified on liver biopsy. Subsequently, the patient had variceal bleeding that necessitated transjugular intrahepatic portosystemic shunt placement. A few similar cases of NCPH occurring in the setting of MG have been previously reported, suggesting that the immunological mechanisms involved in the pathogenesis of myasthenia may also have contributed to the development of NCPH.

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Section: Discussionmentioning

confidence: 99%

“…Many systemic illnesses and some medications have been associated with NCPH with each one possibly providing a clue to its pathogenesis. Various immunologically mediated and collagen vascular diseases have been associated with NCPH …”

Section: Introductionmentioning

confidence: 99%

Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work

Agrawal

Rahmani

Nakkala

et al. 2013

Hepatology Research

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“…Several reports showed the association of IPH and autoimmune diseases . The autoimmune mechanism is considered to be a pathogenesis of IPH.…”

Section: Discussionmentioning

confidence: 99%

“…IDIOPATHIC PORTAL HYPERTENSION (IPH) is characterized by portal hypertension, splenomegaly and anemia in the absence of cirrhosis or occlusion of the hepatic or extrahepatic portal vein, although pathogenetic processes remain unknown. Recently, several reports have shown the association of IPH and autoimmune diseases . The autoimmune mechanism is considered to be a pathogenesis of IPH.…”

Section: Introductionmentioning

confidence: 99%

Case of idiopathic portal hypertension complicated with autoimmune hepatitis

Abe

Takahashi

Sato

et al. 2013

Hepatology Research

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We report a case of idiopathic portal hypertension (IPH) complicated with autoimmune hepatitis. A 60-year-old woman was admitted to our hospital with esophageal and gastric varices in February 2010. Abdominal ultrasonography and computed tomography showed splenomegaly and collateral veins without evidence of liver cirrhosis. Laboratory examinations and liver biopsy indicated that the esophageal and gastric varices were caused by IPH. She underwent endoscopic injection sclerotherapy and partial splenic embolization. Two years after these therapies, laboratory examinations showed liver dysfunction with elevated levels of aspartate aminotransferase (180 IU/L), alanine aminotransferase (190 IU/L), γ-glutamyl transpeptidase (159 IU/L) and immunoglobulin G (2609 mg/dL). The titer of antinuclear antibodies was 1:320 and its pattern was homogeneous and speckled. Histological examination revealed plasma cell/lymphocyte infiltration and interface hepatitis in the portal tract. Based on these findings, a diagnosis of autoimmune hepatitis accompanied by IPH was made. After treatment with prednisolone (20 mg/day), liver functions were normalized immediately. Overlapping of IPH and AIH is extremely rare, but the present case is interesting considering the etiology of IPH because an autoimmune mechanism is thought to be involved in the pathogenesis of IPH.

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Portal Hypertension in Rheumatic Diseases

Takagi

Ohira

2019

Gastrointestinal and Hepatic Manifestations of Rheumatic Diseases

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A case of idiopathic portal hypertension associated with rheumatoid arthritis

Cited by 9 publications

References 14 publications

Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work

Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work

Case of idiopathic portal hypertension complicated with autoimmune hepatitis

Portal Hypertension in Rheumatic Diseases

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