Abnormal liver function in common variable immunodeficiency disorders due to nodular regenerative hyperplasia

Abstract

SummaryPatients with common variable immunodeficiency disorders are monitored for liver function test abnormalities. A proportion of patients develop deranged liver function and some also develop hepatomegaly. We investigated the prevalence of abnormalities and types of liver disease, aiming to identify those at risk and determine outcomes. The local primary immunodeficiency database was searched for patients with a common variable immunodeficiency disorder and abnormal liver function and/or a liver biopsy. Patterns of liver dysfunction were determined and biopsies reviewed. A total of 47 of 108 patients had deranged liver function, most commonly raised alkaline phosphatase levels. Twenty-three patients had liver biopsies. Nodular regenerative hyperplasia was found in 13 of 16 with unexplained pathology. These patients were more likely to have other disease-related complications of common variable immunodeficiency disorders, in particular non-coeliac (gluten insensitive) lymphocytic enteropathy. However, five had no symptoms of liver disease and only one died of liver complications. Nodular regenerative hyperplasia is a common complication of common variable immunodeficiency disorders but was rarely complicated by portal hypertension.

Adults with common variable immunodeficiency: a single-center experience*

“…There were also positive relationships between the last 2 features and between daily stool frequency and low body weight. Ward et al found similar results in their study suggesting that splenomegaly and hepatomegaly developed either jointly or separately in patients with CVID (23). They also showed a strong association between nodular regenerative hyperplasia of the liver and lymphoproliferation in their patients.…”

“…In Ward et al 's study, abnormal liver function tests (especially for ALP) and hepatomegaly were present together in some patients with CVID and were associated with elevated GGT (23).…”

Non-cirrhotic Intrahepatic Portal Hypertension: Associated Gut Diseases and Prognostic Factors

“…Schistosomiasis [16], toxins/drugs (arsenic [17,18], vitamin A [19], azathioprine [20], 6-thioguanine [21,22]), immune disorders (Felty's syndrome [23], common variable immunodeficiency disorder [24,25]) and myeloproliferative syndromes [26] are associated with NCIPH. The current study suggests that gut disorders are yet another association to be looked for in patients with NCIPH.…”

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

“…Hepatitis with no evidence of viral infection was reported in 43% of patients in two unrelated cohorts (16,17), and raised blood alkaline phosphatase levels were often observed. In most cases, liver biopsy showed mild to moderate periportal lymphocyte infiltration and cholestasis.…”