AUTO IMMUNE HEPATITIS AS A LIVER MANIFESTATION OF COMMON VARIABLE IMMUNODEFICIENCY: A CASE REPORT

Introduction and Objectives

Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by impaired differentiation of B cells with defective immunoglobulin production and paradoxically the development of autoimmune disorders. It is estimated that the prevalence of the liver disease is less than 10%. The most reported is focal nodular hyperplasia and, to a lesser extent, primary biliary cholangitis, primary sclerosing cholangitis and autoimmune hepatitis (AIH).

Objectives

present the case of a 22-year-old male who was admitted for hematemesis. He was diagnosed with immune thrombocytopenia 12 years ago without treatment and common variable immunodeficiency six years ago, currently three years without treatment. No family history of autoimmune or liver diseases. After extraction of the 4th molars, he presented swelling, redness, heat in the left submandibular region, unquantified fever, for which he received antibiotic treatment and non-steroidal anti-inflammatory drugs; 12 hours before admission, he began with hematemesis and hematochezia with data of hemodynamic instability, for which crystalloids are administered, the physical examination presents scleras with jaundice, an edematous, erythematous area with local heat in the left submandibular region, flat, symmetrical abdomen, absence of collateral circulation, Non-painful hepatomegaly, without shifting dullness. Laboratory admission with anemia, thrombocytopenia, cholestatic pattern in liver biochemistry tests, chronic viral infectious processes are ruled out and antibodies are made for autoimmune diseases, ANA 1:1200 is documented, other antibodies negative. Esophagogastroduodenoscopy where large esophageal varices of Baveno and severe portal hypertensive gastropathy (image 1 panel A). Doppler ultrasound reports diffuse liver disease, 14mm portal vein, with the presence of free fluid perihepatic and perisplenic, without biliary obstruction data (image 1 panel B). Percutaneous liver biopsy: fibrosis F2-3 on the scale of Metavir, interface hepatitis, associated with infiltrate lymphoplasmacytic integrating diagnosis of AIH (image 1 panel C).

Discussion

The prevalence of this condition is estimated at 1 in every 50,000 people worldwide. Up to 25% of patients with CVID will have an association with autoimmune diseases, representing the heterogeneous nature of the disease. The presentation of liver disease commonly reported in case series is anicteric cholestasis, and biopsies show evidence of non-cirrhotic portal hypertension. However, the association of CVID and AIH is rare; its diagnosis requires biopsies due to the lack of expression of antibodies in most cases.

Conclusions

In CVID patients with altered liver function tests, the association with autoimmune liver diseases should be ruled out to initiate timely treatment and avoid late complications.

The authors declare that there is no conflict of interest.