부산대학교 도서관

Post-infantile 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, gender, 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 novoPIGCH 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=0.02), higher alkaline phosphatase (ALP) level (mean value 253.3U/L vs 166.3 U/L p=0.03), higher fibrosis stage (stage 3-4: 57.7% vs stage 0-2: 29.6%, p=0.03), more likely to have de novoPIGCH after transplantation (23.1% vs 11.4%, p=0.04), and less likely to have primary autoimmune liver disease etiology (26.9% vs 47.7%, p=0.04). These results indicate 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 novoPIGCH in allografts is associated with poorer survival. Older age, higher ALP, and advanced fibrosis are adverse prognostic factors.