Table 1.

Relationship between nuclear pore complex proteins, antinuclear antibodies giving the “multiple nuclear dots” immunofluorescence pattern, and clinical significance in patients with primary biliary cholangitis

Nuclear structures	Indirect immunofluorescence pattern	Autoantigen targets	Autoantibodies in PBC
Nuclear structures	Indirect immunofluorescence pattern	Autoantigen targets	Prevalence (%)	Specificity	Worse prognosis
Nuclear Bodies	MND^a	Sp100	17–41	High	To be confirmed
		PML	19	High	No
		Sp140	15	High	No
		Sp140L	1.5	Not assessed	Not assessed
		SUMO-1	15	Not assessed	Not assessed
		SUMO-2	42	Not assessed	Not assessed

Sp100 Sp100 nuclear body component, PML promyelocytic leukemia protein, Sp140 Sp140 nuclear body component, Sp140L Sp140L protein, MND multiple nuclear dots pattern, SUMO small ubiquitin-related modifiers

^aMND pattern detected by indirect immunofluorescence using fluorescein-conjugate anti-human total Ig and anti-human IgG subclasses (IgG1, IgG2, IgG3, IgG4) as secondary antibody was found in 16% and 31%, respectively [1, 2]