A patient with congenital hepatic fibrosis, after living-donor liver transplantation, developed a systemic IgG4-related disease following infection with SARS-CoV-2

Introduction Congenital hepatic fibrosis (CHF) is an autosomal recessive disease characterized by hepatosplenomegaly and portal hypertension. IgG4-related disease is considered a systemic and inflammatory syndrome characterized by enlargement of involved organs, elevated IgG4, dense lymphoplasmacytic infiltrate, and fibrosis. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related coronavirus disease 2019 (COVID-19) is associated with significant morbidity and mortality in liver disease. While immunosuppression after liver transplantation (LT) may attenuate inflammatory response to COVID-19, it may also increase virological injury and prolong viral shedding.

Case report A 23-year-old Caucasian male, diagnosed with CHF in 1998, underwent living-donor LT after multiple bleeding complications. In March 2020, patient was hospitalized with SARS-CoV-2 infection. In December 2020, MRI demonstrated a progressive, elongated tissue plus retroperitoneally along the abdominal aorta to the common iliac artery. Pathologically enlarged lymph nodes were detected (mesenteric, peritoneal, iliac, and inguinal). Whole-body PET confirmed these findings matching to a post-transplant lymphoproliferative disease in 2021. However, EBV-DNA and skewed kappa:lambda immunoglobulin free light chain ratio in peripheral blood were not detectable. Colonoscopy with biopsies demonstrated > 40 positive plasma cells/hpf rich in IgG4-immunostaining. Biopsy of retroperitoneal lymph nodes revealed IgG4-positive plasma cells. In addition, highly elevated IgG4 with 45’300 mg/l along with an IgG of 53’500 mg/l, highly elevated sIL2 receptor with 2’246 U/ml, and increased eosinophils with 11.8 % were present. Treatment of this underlying IgG4-RD with rituximab is scheduled.

Conclusion Diagnosis of IgG4-RD is challenging. This disease may also occur despite immunosuppressive therapy, in our case, probably induced by the coronavirus.

Publication History

Article published online:
26 January 2022