Role of autoantibodies in the clinical management of primary biliary cholangitis

Table 1 Autoantibodies detected in primary biliary cholangitis and their clinical significance

Autoantibodies	Method	Prevalence	Specificity	Diagnostic relevance	Clinical significance
ΑΜΑ	IIFL, ELISA, Blot	90%-95%. Varies according to method used	High (90%)	Yes	AMA presence and titer not associated with severity of PBC or prognosis of PBC
Antinuclear antibodies				Yes
Against nuclear envelope antigens: Anti-sp100; anti-PML; anti-sp140; anti-SUMO	IIFL (MND pattern), ELISA	30%-50%	Very high (approximately 98%)	Useful in AMA-negative patients	Anti-sp100 is associated with more severe disease, faster disease progression and worse outcome
Against nuclear pore complex antigens: Anti-gp210; anti-np62; anti-LBR	IIFL (Rim-like perinu-clear pattern; RL/M), ELISA, Immunoblot	30%-50%	Very high. Up to 100%	Useful in AMA-negative patients	Anti-gp210 is associated with more severe disease and worse outcome
Anticentromere antibodies	IIFL	10%-30%	Low	No	Limited data on their role as prognostic factor for complications related to portal hypertension
Anti-KLHL12 antibodies	ELISA	22%-36%	Very high	Yes. Useful in AMA-negative pts	Anti-HK1 is associated with worse outcome (needs validation)
Anti-HK-1 antibodies	Immunoblot

sp100: Nuclear body speckled 100 kDa; sp140: Nuclear body speckled 140 kDa; SUMOs: small ubiquitin-related modifiers; PML: Promyelocytic leukemia proteins; LBR: Lamin B receptor antibody; KLHL12: Anti-kelch-like 12 protein; HK-1: Anti-hexokinase 1; ΑΜΑ: Anti-mitochondrial antibodies; MND: Multi nuclear dot pattern; ELISA: Enzyme-linked immunosorbent assay; IIFL: Indirect immunofluorescence; PBC: Primary biliary cholangitis.