Liver stiffness assessment by transient elastography suggests high prevalence of liver involvement in common variable immunodeficiency

Crescenzi, Ludovica; Pecoraro, Antonio; Fiorentino, A.; Poto, Remo; Varricchi, Gilda; Rispo, Antonio; Morisco, Filomena; Spadaro, Giuseppe

doi:10.1016/j.dld.2019.05.016

Cited by 19 publications

(42 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This ultrasound-based technique using a hand-held ultrasound probe allows non-invasive measurement of liver stiffness. Interpretation of generated data depends on the underlying disease-pathology [23][24][25][26][27]; however, transient elastography has previously been demonstrated to be of diagnostic value for CVID patients, particularly in those at risk for portal hypertension [20,28]. ROC analysis of the LSM was performed, and the optimal cutoff for determination of portal hypertension was calculated by the Youden Index.…”

Section: Transient Elastographymentioning

confidence: 99%

“…With regard to laboratory markers of liver disease in CVID, especially elevations in alkaline phosphatase (ALP), bilirubin and transaminases have been described [18,19]. Abdominal ultrasound and magnetic resonance imaging as well as transient elastography (FibroScan®) can be used to assess structural changes in the liver as well as signs of portal hypertension [20][21][22]. There is, however, so far no standard in the field with regard to which parameters should be assessed in CVID patients to determine whether the patient suffers from portal hypertension.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

et al. 2022

View full text Add to dashboard Cite

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.

show abstract

Section: Transient Elastographymentioning

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

View full text Add to dashboard Cite

show abstract

“…This has been used to assess liver involvement without exposing the patient to the risk of infection or bleeding from a biopsy procedure. 49 However, biopsy is needed in many cases to ascertain the etiology of liver damage. 50 Biopsies typically reveal nonspecific portal and lobular inflammation, hepatitis, lymphocyte infiltration without plasma cells, granulomas, fibrosis, macrovesicular steatosis, and neogenesis of biliary ducts.…”

Section: Hepatic and Splenic Evaluationmentioning

confidence: 99%

State-of-the-art diagnostic evaluation of common variable immunodeficiency

Lee

Gereige

Maglione

2021

Annals of Allergy, Asthma & Immunology

View full text Add to dashboard Cite

To summarize the current understanding of diagnostic and postdiagnostic evaluation of common variable immunodeficiency (CVID). Data Sources: PubMed Central database. Study Selections: Original research articles and review articles from 2015 to 2020 including seminal articles that shaped the diagnostic and postdiagnostic evaluation of CVID were incorporated. This work focuses on initial diagnosis of CVID, genetic evaluations, and postdiagnostic assessment of respiratory, gastrointestinal, and hepatobiliary diseases including spleen and lymph node enlargement. Results: CVID presents not only with frequent infections but also with noninfectious complications such as autoimmunity, gastrointestinal disease, chronic lung disease, granulomas, liver disease, lymphoid hyperplasia, splenomegaly, or malignancy. The risk of morbidity and mortality is higher in patients with CVID and noninfectious complications. Detailed diagnostic approaches, which may incorporate genetic testing, can aid characterization of individual CVID cases and shape treatment in some instances. Moreover, continued evaluation after CVID diagnosis is key to optimal management of this complex disorder. These postdiagnostic evaluations include pulmonary function testing, radiologic studies, and laboratory evaluations that may be conducted at frequencies determined by disease activity. Conclusion: Although the diagnosis can be achieved similarly in all patients with CVID, those with noninfectious complications have distinct concerns during clinical evaluation. State-of-the-art workup of CVID with noninfectious complications typically includes genetic analysis, which may shape precision therapy, and thoughtful application of postdiagnostic tests that monitor the presence and progression of disease in the myriad of tissues that Key MessagesRecent advancements in our understanding of common variable immunodeficiency (CVID) pathogenesis now shape diagnostic and longitudinal evaluation of this disease.Workup of CVID involves thoughtful application of testing to identify and monitor the presence and progression of disease in the myriad of tissues that may be affected.Genetic testing is potentially useful in CVID, particularly those with noninfectious complications for which it may shape treatment.Given the extensive heterogeneity of CVID, not all with this diagnosis should be followed with an identical approach. Those with complicated courses warrant closer, more frequent monitoring tailored to their specific manifestation of CVID. Substantial gaps in our knowledge of CVID diagnostic evaluation persist and offer potential to improve identification and management of this primary immunodeficiency if elucidated.

show abstract

“…TE allows estimating the degree of liver fibrosis through the assessment of liver stiffness and depends on vibration generating machine to apply vibrations to the liver and then obtain the propagation velocity of shear wave (130). We recently investigated liver involvement in a cohort of CVID adult patients by means of ultrasound based TE, finding that 33.8% patients presented increased liver stiffness values ranging from moderate fibrosis to cirrhosis (40). Interestingly, TE values were correlated with ALP and γGT values, spleen longitudinal diameter and peripheral blood counts.…”

Section: Diagnostic Work-upmentioning

confidence: 99%

“…Raised alkaline phosphatase (ALP) levels were observed in 43.5% CVID patients of a 2008 cohort study (35), while histologically proved liver disease was demonstrated in a smaller proportion of subjects in two other studies (9.1% and 9.3%, respectively) (38,39). Our research group recently reported a liver disease prevalence of 33.8% in a cohort of 77 adult CVID patients in whom liver involvement was assessed through the measurement of liver stiffness by ultrasound-based transient elastography (TE) (40). Finally, 79% CVID patients referred to a United Kingdom Hepatology Center displayed laboratory, imaging and/or histological signs of liver disease (36).…”

Section: Introductionmentioning

confidence: 97%