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Published on: May 29, 2020
Postinfantile Giant Cell Hepatitis in Native and Allograft Livers: A Multi-Institutional Clinicopathologic Study of
Jingjing Jiao1, Ksenia Chezar2, Xuefeng Zhang2
1Department of Pathology, Yale University School of Medicine, New Haven, Connecticut.
Insights
Postinfantile giant cell hepatitis (PIGCH) is a rare liver condition with varied causes. Autoimmune liver disease offers better survival, while de novo PIGCH in transplants indicates poorer outcomes.
Area of Science:
- Hepatology
- Pathology
- Transplantation
Background:
- Postinfantile giant cell hepatitis (PIGCH) presents a rare pattern of liver injury in adults.
- Etiologies and clinical outcomes for PIGCH are highly variable.
Purpose of the Study:
- To define the clinicopathologic characteristics of patients with PIGCH.
- To identify factors associated with survival in PIGCH patients.
Main Methods:
- Multi-institutional retrospective study of 70 PIGCH cases.
- Review of pathological features (fibrosis, inflammation, giant cells) and clinical data (etiology, labs, follow-up).
Main Results:
- Common etiologies included autoimmune liver diseases (40%), unknown (13%), viral (11%), and de novo PIGCH in allografts (16%).
- Survival was better with autoimmune liver disease etiology (47.7% vs 26.9%) and poorer in de novo PIGCH post-transplant (23.1% vs 11.4%).
- Adverse prognostic factors included older age, elevated alkaline phosphatase, and advanced fibrosis.
Conclusions:
- PIGCH is a rare liver injury pattern with diverse causes and outcomes.
- Autoimmune liver disease is linked to better PIGCH survival, whereas de novo PIGCH in liver allografts is associated with worse survival.
- Older age, high alkaline phosphatase, and advanced fibrosis are poor prognostic indicators for PIGCH.
Abstract:
Postinfantile giant cell hepatitis (PIGCH) is a rare hepatitis pattern in adults with variable etiologies and clinical outcomes. We conducted a multi-institutional retrospective study to define the clinicopathologic characteristics of patients with PIGCH. A total of 70 PIGCH cases were identified and reviewed for pathological features, including fibrosis, cholestasis, inflammation, steatosis, necrosis, and apoptosis, as well as the distribution of giant cells and the maximum number of giant cells per high-power field. Demographic and clinical data, including age, sex, laboratory results, etiologies, and follow-up results, were recorded. Among the 70 cases, 40% (28/70) were associated with autoimmune liver diseases, followed by 9 (13%) with unknown etiology, 8 (11%) with viral infection, 5 (7%) with medications, 5 with combined etiologies, and 4 (6%) with malignancies (mostly chronic lymphocytic leukemia). Notably, another 16% were de novo PIGCH in liver allografts, most of which occurred after a rejection event. During follow-up, 26 (37%) patients died of the disease and 44 (63%) were alive. Deceased patients were characterized by older age (mean age, 54.9 vs 45.5 years; P = .02), higher alkaline phosphatase level (mean value, 253.3U/L vs 166.3 U/L; P = .03), higher fibrosis stage (stage 3-4 vs stage 0-2, 57.7% vs 29.6%; P = .03), being more likely to have de novo PIGCH after transplantation (23.1% vs 11.4%; P = .04), and being less likely to have primary autoimmune liver disease etiology (26.9% vs 47.7%; P = .04). These results indicate that PIGCH is a rare pattern of liver injury associated with different etiologies and variable clinical outcomes. Autoimmune liver disease with PIGCH is associated with better survival, whereas de novo PIGCH in allografts is associated with poorer survival. Older age, higher alkaline phosphatase level, and advanced fibrosis are adverse prognostic factors.
