Hepatic Rupture in the Setting of Amyloidosis: A Systematic Review

Hepatic rupture in amyloidosis is a rare, highly lethal, and poorly understood condition. This study aimed to summarize the clinical features, therapeutic options, and prognosis of this rare disease.

A literature search of the PubMed, Embase, Scopus, and Web of Science databases for relevant full-text studies was performed. Case reports directly addressing hepatic rupture in amyloidosis were retrieved. Additional studies were identified by reviewing the reference lists of the retrieved studies. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines for data extraction and quality assessment were applied.

Thirty-one patients with hepatic rupture with amyloidosis (14 males [45.2%], 17 females [54.8%]; mean age: 49 years) were identified. Most patients had right lobe involvement (52.9%), followed by involvement of both lobes (29.4%), and left lobe involvement (17.6%). The 31 patients were treated with supportive therapy (54.8%), surgery (41.9%), angiographic embolization (35.5%), chemotherapy (29.0%), or liver transplantation (12.9%) and exhibited a high in-hospital mortality rate (45.2%).

Hepatic rupture in the setting of amyloidosis is extremely ominous and is associated with high mortality rates. The combination of supportive therapy with hepatic artery embolization, surgery, chemotherapy, and liver transplantation has contributed to a decrease in the mortality rate of this disease.