Hepatic involvement in Wegener's granulomatosis: a case report

Abstract

Introduction: We report the case of a 58-year-old Caucasian Greek man who presented with dry cough, fever, bilateral alveolar infiltrates and acute hepatitis.

Case presentation: After a lung biopsy, the patient was diagnosed with Wegener's granulomatosis. The diagnosis was supported by the presence of anti-proteinase-3 anti-neutrophil cytoplasmic antibodies. A liver biopsy demonstrated the presence of mild non-specific lobular hepatitis and periodic acid-Schiff positive Lafora-like inclusions in a large number of his liver cells. The patient was treated with prednisone and cyclophosphamide, which was followed by subsequent remissions of chest X-ray findings and liver function studies.

Conclusion: What makes this case worth reporting is the coexistence of liver inflammation with a biochemical profile of severe anicteric non-viral, non-drug induced hepatitis coinciding with the diagnosis of Wegener's granulomatosis. Our paper may be the first report of hepatic involvement in a patient diagnosed with Wegener's granulomatosis. The aetiological link between the two diseases is supported by the reversion of hepatitis after the immunosuppression of Wegener's granulomatosis. We favor the hypothesis that hepatic vasculitis may be the cause of acute hepatocellular necrosis.

Figure 1

Lung biopsy (fine needle biopsy), periodic acid Schiff stain ×400 magnification.

Figure 2

Lung biopsy (fine needle biopsy), hematoxylin and eosin stain ×400 magnification.

Figure 3

(A) Liver biopsy showing focal necrosis with neutrophils and sinusoidal reaction, hematoxylin and eosin stain ×400 magnification [R1]. (B) Liver biopsy showing Lafora body-like inclusions, hematoxylin and eosin stain ×400 magnification [R2].

Figure 4

Liver function tests in the course of time (0 on the X axis stands for the first day of admission to our department).