A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review

Takagi, Kenji; Nishio, Shinichiro; Akimoto, Kimiko; Yoshino, Takumi; Kawai, Shinichi

doi:10.3109/s10165-006-0479-1

Cited by 11 publications

(8 citation statements)

References 7 publications

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“…However, it is widely accepted that autoimmune diseases (especially connective tissue diseases) increases the prevalence of IPH in certain patient groups. The most important IPH associated diseases are mixed connective tissue disease (6 cases [6] ), systemic sclerosis (20 cases [7] ) and systemic lupus erythematosus (16 cases [8] ). In addition, a Japanese survey [9] found that hypergammaglobulinemia is the most common presenting autoimmune dysfunction (26% of all included IPH patients), along with chronic thyroiditis as the most common autoimmune disease.…”

Section: Autoimmunity Theorymentioning

confidence: 99%

Clinical characteristics of idiopathic portal hypertension

Harmancı

Bayraktar²

2007

WJG

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Idiopathic portal hypertension is one of the interesting causes of portal hypertension. Even in very developed medical centers, this disorder is still one of the most important misdiagnoses of clinical practice. To inexperienced physicians, presenting esophageal varices and upper gastrointestinal bleeding usually prompt an unfortunate diagnosis of cirrhosis. A heterogenous clinical presentation and progression of this disorder should be recognized by physicians, and management should be directed towards some specific problems confined to this disorder. Although a genetic basis and other factors are implicated in its pathogenesis, exact underlying mechanism(s) is (are) unknown. In this review, we discuss the heterogeneity of idiopathic portal hypertension, its etiopathogenesis, clinical presentation and management issues. With the expectation of an excellent prognosis, a practicing gastroenterologist should be aware that "not all varices mean cirrhosis".

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Section: Autoimmunity Theorymentioning

confidence: 99%

Clinical characteristics of idiopathic portal hypertension

Harmancı

Bayraktar²

2007

WJG

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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%

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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“…In some patients we can find HPS in association with immunological disorders such as immunodeficiency [8], rheumatoid arthritis, systemic lupus erythematosus, scleroderma, celiac disease, Sjögren's Syndrome (SS) [9]. Very few examples of association between HPS and Systemic Sclerosis (SSc) are described in the literature: In 2005 Kogawa et al 10] presented a rare case of a 72 years-old Japanese woman with HPS associated with SSc and SS; and in 2006 Takagi et al report a similar case with portal hypertension and SSc [11]. In 2014 the case of a female patient with atypical hepatic changes due to HPS with limited systemic sclerosis was referred in Brazil [12].…”

Section: Abstract: Idiopathic Non-cirrhotic Portal Hypertension (Incph;mentioning

confidence: 99%

Hepatoportal Sclerosis in patient with Systemic Sclerosis and Sjögren’s Syndrome: a rare association

Soardo¹,

Orsaria²,

Scatà³

et al. 2021

J Clin Images Med Case Rep

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We present the case of a 51 years-old woman who was referred to our Liver Unit for suspected dysmetabolic liver disease. She had a previous diagnosis of Systemic Sclerosis and Sjögren’s Syndrome and had altered hepatic enzymes with a positive anti-Nuclear Antibodies centromeric pattern, anti-Cardiolipin Antibodies and anti b2 Glycoprotein I Antibodies. Despite complete liver assessment and disease staging was negative, the clinical course was complicated by the development of anemia, due to esophageal varices bleeding associated with worsening splenomegaly. Liver biopsy was key for reaching the diagnosis as it showed portal tracts with fibrous expansion, ductular proliferation and focal lympho-granulocytic infiltrate, reduced caliber of portal vessels, hypoplastic portal tracts, focal herniation, aberrant microvasculature and positive endothelial CD34 staining. Having ruled out any other cause of portal hypertension such as cirrhosis, blood diseases, occlusion of the hepatic and portal veins, etc., we finally concluded that the portal hypertension was due to hepatoportal sclerosis associated with Systemic Sclerosis and Sjögren’s Syndrome. Keywords: Idiopathic Non-Cirrhotic Portal Hypertension (INCPH; Hepatoportal Sclerosis (HPS); Systemic sclerosis (SSc); Sjögren’s Syndrome (SS).

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A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review

Cited by 11 publications

References 7 publications

Clinical characteristics of idiopathic portal hypertension

Clinical characteristics of idiopathic portal hypertension

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

Hepatoportal Sclerosis in patient with Systemic Sclerosis and Sjögren’s Syndrome: a rare association

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