International multicenter experience of transjugular intrahepatic portosystemic shunt implantation in patients with common variable immunodeficiency

Globig, Anna-Maria; Heeg, Maximilian; Larsen, Carsten Schade; Ferreira, Rúben; Kindle, Gerhard; Goldacker, Sigune; Strohmeier, Valentina; Silva, Susana L.; Cunningham‐Rundles, Charlotte; Quinti, Isabella; Thimme, Robert; Bettinger, Dominik; Schultheiß, Michael; Warnatz, Klaus

doi:10.1016/j.jaip.2021.02.056

Cited by 7 publications

(8 citation statements)

References 9 publications

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“…While infectious complications, lung function, cytopenias, gastrointestinal involvement, and immunoglobulin levels are more closely monitored by clinicians, affection of the liver with development of portal hypertension is a severe complication that is usually recognized only at advanced stages. Importantly, diagnostic markers and therapeutic approaches in fibrotic liver disease and portal hypertension due to CVID are insufficiently defined [ 13 ]. Chronic liver disease has been reported to occur in ~ 12% of CVID patients seen in tertiary treatment centers, and occurrence of liver disease is associated with increased mortality [ 11 , 12 , 14 ].…”

Section: Introductionmentioning

confidence: 99%

“…Underlying pathologies comprise granulomatous inflammation, autoimmune hepatitis, and most commonly nodular regenerative hyperplasia (NRH) [ 15 ]. While liver function may be still preserved, the major complication of NRH is portal hypertension with development of ascites and/or esophageal varices that can ultimately cause potentially fatal upper gastrointestinal bleeding [ 13 , 16 , 17 ].…”

Section: Introductionmentioning

confidence: 99%

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Evaluation of Laboratory and Sonographic Parameters for Detection of Portal Hypertension in Patients with Common Variable Immunodeficiency

et al. 2022

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Timely detection of portal hypertension as a manifestation in a subgroup of patients with common variable immunodeficiency (CVID) represents a challenge since it is usually not associated with liver cirrhosis. To identify relevant markers for portal hypertension, we evaluated clinical history, laboratory parameters, and abdominal ultrasound including liver elastography and biomarkers of extracellular matrix formation. Twenty seven (6%) of 479 CVID patients presented with clinically significant portal hypertension as defined by either the presence of esophageal varices or ascites. This manifestation occurred late during the course of the disease (11.8 years after first diagnosis of CVID) and was typically part of a multiorgan disease and associated with a high mortality (11/27 patients died during follow up). The strongest association with portal hypertension was found for splenomegaly with a longitudinal diameter of > 16 cm. Similarly, most patients presented with a liver stiffness measurement (LSM) of above 6.5 kPa, and a LSM above 20 kPa was always indicative of manifest portal hypertension. Additionally, many laboratory parameters including Pro-C4 were significantly altered in patients with portal hypertension without clearly increasing the discriminatory power to detect non-cirrhotic portal hypertension in CVID. Our data suggest that a spleen size above 16 cm and an elevated liver stiffness above 6.5 kPa should prompt further evaluation of portal hypertension and its sequelae, but earlier and better liquid biomarkers of this serious secondary complication in CVID are needed.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Evaluation of Laboratory and Sonographic Parameters for Detection of Portal Hypertension in Patients with Common Variable Immunodeficiency

et al. 2022

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“… [90] , [91] , [92] In a retrospective study, Globig et al. 93 collected data from a cohort of 13 patients with CVID who underwent TIPS in clinical centres in Europe and North America. NRH as an underlying liver disease was observed in nine patients.…”

Section: Current Treatment Options In Cvid-related Liver Diseasementioning

confidence: 99%

“… 47 Impairment of the liver-gut axis could also contribute to the high rate of sepsis after TIPS, as suggested by some. 93 The liver tissue acts as a gatekeeper between the systemic circulation and pathogens in the gut, 97 , 98 however, its ability to perform this role could be impaired by the portosystemic shunting that is inherent to TIPS. Additionally, microbial dysbiosis in CVID 62 and microbial translocation could further increase the risk of sepsis.…”

Section: Current Treatment Options In Cvid-related Liver Diseasementioning

confidence: 99%

Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)

Baumert,

Shih,

Chung

2023

JHEP Reports

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“…TIPS implantation reduces portal vein pressure, but these patients are at a significantly higher risk of developing infections after the intervention. Therefore, TIPS implantation should only be performed after critical evaluation of the patient and after interdisciplinary discussions [38].…”

Section: No Evidence-based Indications For Transjugular Intrahepatic ...mentioning

confidence: 99%

Implantation of transjugular intrahepatic portosystemic shunt (TIPS): indication and patient selection

Bettinger

Thimme

Schultheiß

2022

Current Opinion in Gastroenterology

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Purpose of reviewThis review summarizes indications and contraindications for implantation of transjugular intrahepatic portosystemic shunt (TIPS). Further, patient selection strategies are discussed.Recent findings TIPS implantation is a highly effective treatment for portal hypertension. Main indications are ascites and variceal bleeding in patients with liver cirrhosis. There is growing evidence that early TIPS implantation after variceal bleeding is associated with an improved survival (preemptive TIPS). Preliminary data also suggest that an analogous concept of early TIPS implantation may be beneficial for patients with ascites. Further, well-selected patients with acute or chronic nonmalignant portal vein thrombosis can be effectively treated with TIPS implantation. In contrast, there is generally no recommendation for TIPS implantation in patients with hepatic veno-occlusive disease, noncirrhotic portal hypertension or prior before surgery to avoid complications of portal hypertension. Apart from evidencebased patient selection, the newly developed FIPS score can be an objective component in decisionmaking.

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International multicenter experience of transjugular intrahepatic portosystemic shunt implantation in patients with common variable immunodeficiency

Cited by 7 publications

References 9 publications

Evaluation of Laboratory and Sonographic Parameters for Detection of Portal Hypertension in Patients with Common Variable Immunodeficiency

Evaluation of Laboratory and Sonographic Parameters for Detection of Portal Hypertension in Patients with Common Variable Immunodeficiency

Management of liver disease and portal hypertension in common variable immunodeficiency (CVID)

Implantation of transjugular intrahepatic portosystemic shunt (TIPS): indication and patient selection

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