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

In 30% of patients, common variable immunodeficiency (CVID) is associated with noninfectious manifestations such as enteropathy, interstitial lung disease, granuloma, splenomegaly, autoimmunity, malignancy, and liver disease.1 Liver disease typically involves nodular regenerative hyperplasia (NRH), granulomatous hepatopathy, and less frequently, autoimmune hepatitis.2,3 Although liver function is often preserved, most patients with NRH present with portal hypertension associated with the development of ascites and esophageal varices that can cause life-threatening upper-gastrointestinal bleeding.4 Variceal bleeding and recurrent ascites are major indications for the implantation of a transjugular intrahepatic portosystemic shunt (TIPS) in patients with liver cirrhosis and portal hypertension.5 Whether TIPS implantation is also a feasible option to treat CVID patients who develop complications of portal hypertension is currently addressed only in individual case reports.6 We therefore retrospectively collected the clinical data of 13 CVID patients from five clinical centers in Europe and North America who were treated with TIPS implantation. Nine patients experienced NRH; two had granulomatous liver disease, one had cryptogenic cirrhosis, and one had hepatitis C–related liver cirrhosis (Table I). In the general TIPS population, liver enzymes have been shown to increase within 2 to 5 days of TIPS implantation in patients with acute hepatic decompensation, but to normalize within a month.7 Six of 13 patients died during the follow-up (Table II): two patients died of septic shock and multiorgan failure as a result of spontaneous bacterial peritonitis with detection of Escherichia coli; another patient died of septic shock owing to smoldering mediastinitis after esophagus perforation with intestinal translocation and detection of E coli, Enterobacter cloacae, Stenotrophomonas maltophilia, and Enterococcus faecium. [...]there was a highly relevant risk for death owing to sepsis even up to almost 4 years after implantation, which requires better ways of prevention, possibly by adding antibiotic prophylaxis.Online Repository ID Sex First diagnosis of CVID (age, y) First manifestation of CVID (age, y) Genetics Gastrointestinal disease Spleen diameter before TIPS, cm First diagnosis of liver disease (age, y Etiology of liver disease Indication for TIPS Age at TIPS, y Freiburg-1 F 39 6 Unknown Previous bacterial GI infection, chronic norovirus infection Splenectomy 2