Long-term Follow-up of Severe Eosinophilic Hepatitis: A Rare Presentation of Hypereosinophilic Syndrome

Abstract

Idiopathic hypereosinophilic syndrome (HES) is a rare, heterogeneous disorder characterized by a strikingly high eosinophil count (>1,500 cells/μL), over a long period of time (>6 months), with end organ damage. We present a 60-year-old patient with idiopathic HES with isolated liver involvement, a rare systemic disease and a rare solid organ involvement. The patient had a thorough investigational work up until HES was established, including liver biopsy. He needed intensive immunosuppressive treatment at first with steroids, then with azathioprine in conjunction with a low dose of steroids. After 16 years of follow-up, the patient showed no evidence of liver dysfunction. To the best of our knowledge, this is the longest follow-up for a patient with HES-associated chronic hepatitis. Our observation suggests that, with appropriate treatment, liver involvement in HES may be well controlled without deterioration to advanced liver failure.

Figure 1

Liver Biopsy at First Evaluation and Repeated Biopsy Four Years Later. A liver needle biopsy taken within the first evaluation showed preserved lobular architecture (A, HE 4×) and mild portal inflammation with several eosinophils seen in the infiltrate (B, HE 40×). Masson trichrome stain showed focal bridging fibrosis (C, trichrome 4×). A second biopsy taken four years later showed portal inflammation (D, HE 4×) with fewer eosinophils in the infiltrates (E, HE 40×) and no change in fibrosis (F, trichrome 4×). White arrowheads indicate eosinophils.

Figure 2

Eosinophilic Count, Liver Function Tests, and Enzymes from Presentation to Date. ALP, alkaline phosphatase (U/L); ALT, alanine transaminase (U/L); AST, aspartate transaminase (U/L); Bil Direct, bilirubin direct (mg/dL); Bil Total, bilirubin total (mg/dL); Eos, eosinophils (×10³/μL); GGT, gamma-glutamyl transpeptidase (U/L); Max, worst value during hospitalization; Spont, spontaneous improvement; Steroids & Aza, concomitant treatment of steroids an azathioprine; Tapering, tapering down of steroids.