Autoimmunity in Common Variable Immunodeficiency: Case report

Introduction: Common variable immunodeficiency (CVID) is a disease characterized by deficient antibody production although, it is paradoxically associated with several autoimmune disorders. The most common causes of liver disease in patients with CVID are nodular regenerative hyperplasia, granulomatous infiltration of the liver. We present a case of autoimmunity as the only manifestation in a patient with CVID without recurrent infections.

Case presentation: A 24-year-old male with a history of ITP at 10 years of age, treated with prednisolone for 4 months, platelet count lower than 1,000 and multiple transfusions of platelets, relapse at 15 years of age. He presented with hematemesis and melena of 12 hours of evolution, syncope, headache. BP 80/40, HR 140 bpm, hydric resuscitation was started and esophagoduodenoscopy was performed, reporting large esophageal varices of Baveno, they were ligated, Doppler ultrasound was performed with data of chronic liver disease. Complementary laboratory studies and liver biopsy were performed, which reported mild interface hepatitis with lobular lymphocytic infiltrate.

IgG 137 mg/dl
IgM 17.8 mg/dl
IgA 18.4 mg/dl
IgE 16.7 UI/ml
ANA negative
anti-SMA >1:40
anti-LKM negative
Flow cytometry: B lymphocytes (CD19, CD20): 9%, fulfilling criteria for CVID and autoimmune hepatitis (AIH).

Discussion: This case reaffirms that detection of autoimmune disease in CVID may be challenging, as the altered humoral response of these patients often results in their inability to develop autoimmune markers (IgG may be low, reflecting disease, or high in the context of recent IVIG treatment). Because of the above considerations, the broader IAIHG criteria rather than the abbreviated criteria were used to diagnose AIH with a score of 15, and the diagnosis was obtained.

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