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

• DOI: 10.1007/s10875-022-01319-0
• Publication Date: 2022-07-12T16:07:57Z
• Authors: Anna-Maria Globig; Valentina Strohmeier; Rambabu Surabattula; Diana J. Leeming; Morten A. Karsdal; Maximilian Heeg; Gerhard Kindle; Sigune Goldacker; Caroline von Spee...; Michele Proietti; Birke Bausch; Dominik Bettinger; Michael Schultheiß; Robert Thimme; Detlef Schuppan; Klaus Warnatz
• Keywords: Common Variable Immunodeficiency; Medical microbiology

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