Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia

Authors Petros Doumtsis, Theodora Oikonomou, Ioannis Goulis, Kaliopi Zachou, George Dalekos, Evangelos Cholongitas.


Autoimmune hepatitis (AIH) is a progressive, chronic liver disease characterized by unresolving hepatocellular inflammation of autoimmune origin. The clinical spectrum may vary from asymptomatic presentation, to non-specific symptoms such as fatigue, arthralgias, nausea and abdominal pain, to acute severe liver disease. AIH is characterized by the presence of interface hepatitis and portal plasma cell infiltration on histological examination, hypergammaglobulinemia, and positive autoantibodies. AIH is associated with other autoimmune diseases and its course is often accompanied by various non-specific hematological disorders. However, the coexistence of autoimmune neutropenia (AIN) is infrequent. We present a case of a female patient diagnosed with type 1 AIH and agranulocytosis on presentation. A diagnosis of AIN was established, based on the patient's sex, the underlying liver disease, the absence of alternative causes, the presence of atypical anti-neutrophil cytoplasmic antibodies in patient's serum and the favorable and dosedependent treatment of both pathologic entities with corticosteroids and mycophenolate mofetil.

Keywords Agranulocytosis, autoimmune neutropenia, autoimmune hepatitis, anti-neutrophil cytoplasmic antibodies, chronic hepatitis, corticosteroids, mycophenolate mofetil

Ann Gastroenterol 2018; 31 (1): 123-126

Case Report