Early histopathologic changes in primary biliary cholangitis: does ‘minimal change’ primary biliary cholangitis exist? A pathologist’s view

Abstract

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an autoimmune, slowly progressive, cholestatic liver disease characterized by nonsuppurative destructive cholangitis, and interlobular bile duct destruction. Necroinflammatory activities of the hepatic parenchyma and limiting plates of milder form along with late liver fibrosis may develop. Serum liver tests include elevated serum alkaline phosphatase along with a positive antimitochondrial antibody (AMA) in nearly 95% of patients. Liver biopsies are an important confirmatory and staging tool and are additionally very helpful when AMA is negative. More specifically, the earliest changes in liver biopsy suspicious for PBC can be detected, namely loss of the canals of Hering (CoH), as proposed by various authors recently. CoH loss has been described as an early feature of PBC. We focus on early histologic features of PBC, investigating through the literature the possible role of ‘minimal change’ supporting the clinical diagnosis of PBC, even in the absence of characteristic granulomatous duct destructive lesions.

Inflammatory Myopathy Associated with Anti-mitochondrial Antibody-negative Primary Biliary Cholangitis Diagnosed by a Liver Biopsy

“…Although they are regarded as serological hallmarks of PBC, they are not detectable in 10-22% of PBC patients when tested by IIF, nor in 5-10% even when more accurate immunoassays, such as a CLEIA, are used (11). The early phase of PBC may help explain the AMA negativity (12). However, the presence of AMAs can reportedly predate histological and biochemical manifestations by several years (13).…”

Granulomatous liver diseases

“…Granulomas are usually evident in early disease, characterized by portal inflammation, sometimes called the florid duct lesion stage. 62 The florid duct lesion is a diagnostic histological feature of PBC and represents a damaged bile duct, surrounded by lymphocytes and plasma cells, with adjacent granulomas. Granulomas are mostly epithelioid, non-necrotizing, and are poorly formed, with many infiltrating lymphocytes that disrupt the structure.…”