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:10.1007/s10165-006-0456-8

Cited by 7 publications

(8 citation statements)

References 24 publications

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“…Recent studies have shown that IPH is associated with many autoimmune diseases, such as systemic sclerosis 15 , systemic lupus erythaematosus 16 and rheumatoid arthritis 9 . These results, combined with our own, indicate that IPH may be secondary to inflammation of connective tissue and vasculitis.…”

Section: Discussionmentioning

confidence: 99%

“…The cause of IPH is complicated. The most common cause is certain kinds of drugs, such as thiopurine 1-3 and arsenical salts 4,5 , and other causes include a series of autoimmune diseases, such as Hashimoto's thyroiditis 6,7 , noninfectious hepatitis 7,8 and rheumatoid arthritis 9 . Similar to cirrhosis, IPH can also lead to hepatic failure, splenomegaly, variceal bleeding and ascites 10 , making it difficult to differentiate IPH from cirrhosis.…”

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confidence: 99%

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Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Zhao

Wei

Wang

et al. 2020

Sci Rep

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Idiopathic portal hypertension (IPH) mimics liver cirrhosis in many aspects, and no efficient imaging method to differentiate the two diseases has been reported to date. In this study, the imaging and pathological characteristics were analysed for both IPH and cirrhosis. From January 2015 to March 2019, ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) images and pathological results from 16 IPH and 16 liver cirrhosis patients, as well as imaging results of 16 normal patients as a control group, were retrospectively reviewed. The age of the patients was 39 ± 20 years. There was a significant difference in the mean lumen diameter, wall thickness and ratio of thickness to diameter between the IPH and liver cirrhosis patients in the main and sagittal portal veins (P < 0.05), as well as in the lumen diameter and ratio of thickness to diameter between the IPH and liver cirrhosis patients in the Segment 3 (S3) portal vein (P < 0.05). In IPH patients, the main imaging changes were portal vein wall thickening, stenosis or occlusion, a low enhancement area along the portal vein in the delay phase in contrast-enhanced imaging, and a non-homogeneous change in T1WI. The corresponding pathological changes included interlobular vein thickening, stenosis, occlusion, portal area fibrosis, and atrophy or apoptosis of hepatocytes. The main imaging characteristic of liver cirrhosis was a nodular change in T1WI, and the related pathological change was pseudolobule formation. The imaging characteristics of IPH include thickening of the portal vein vascular wall, stenosis of the portal vein lumen and the absence of diffuse cirrhosis-like nodules. These imaging features have a definite pathological basis and could help make differential diagnoses between IPH and cirrhosis.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Zhao

Wei

Wang

et al. 2020

Sci Rep

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“…Other rare conditions, including NRH, idiopathic portal hypertension, and spontaneous hepatic rupture secondary to vasculitis, have also been reported. 49 , 57 – 60 , 61 RA is generally defined as the combined presence of arthropathy and the rheumatoid factor (RF). However, similarity in extrahepatic manifestations of primary liver disorders might be confusing.…”

Section: Rheumatoid Arthritismentioning

confidence: 99%

Immune Components of Liver Damage Associated with Connective Tissue Diseases

Chentoufi

Serov

Alazmi

et al. 2014

JCTH

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Autoimmune connective tissue diseases are associated with liver abnormalities and often have overlapping pathological and clinical manifestations. As a result, they can present great clinical challenges and evoke questions about diagnostic criteria for liver diseases. Moreover, discriminating between liver involvement as a manifestation of connective tissue disease and primary liver disease can be challenging since they share a similar immunological mechanism. Most patients with connective tissue diseases exhibit liver test abnormalities that likely result from coexisting, primary liver diseases, such as fatty liver disease, viral hepatitis, primary biliary cirrhosis, autoimmune hepatitis, and drug-related liver toxicity. Liver damage can be progressive, leading to cirrhosis, complications of portal hypertension, and liver-related death, and, therefore, must be accurately identified. In this review, we highlight the challenges facing the diagnosis of liver damage associated with connective tissue disease and identify immune mechanisms involved in liver damage associated with connective tissue diseases.

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“…An accompanying autoimmune disease (e. g. systemic lupus erythematosus, rheumatoid arthritis) is not uncommon in IPH patients [10,12]. Babbs et al report a patient with IPH who developed IgA nephropathy and a nephrotic syndrome [17].…”

Section: Case Presentationmentioning

confidence: 99%

Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature

et al. 2010

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BackgroundIdiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings.Case PresentationWe report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy.ConclusionsOur results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH.

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A case of idiopathic portal hypertension associated with rheumatoid arthritis

Cited by 7 publications

References 24 publications

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Imaging and Pathological Features of Idiopathic Portal Hypertension and Differential Diagnosis from Liver Cirrhosis

Immune Components of Liver Damage Associated with Connective Tissue Diseases

Life-threatening hypersplenism due to idiopathic portal hypertension in early childhood: case report and review of the literature

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