Table 18.3
Interpretation of ANA staining
HEp-2 staining patternAntigen(s)Disease associations
Nuclear staining

Homogeneous nuclear/nucleolar

dsDNA, histones, topoisomerase 1, Ku

SLE

Homogeneous nuclear (nucleoli negative)

Histone

SLE, drug-induced SLE

Nuclear membrane

Lamins A, B, C

Autoimmune liver disease anti-phospholipid syndrome leucocystoclastic vasculitis

Nuclear pore (punctate staining)

gp210 pore protein, lamin B receptor

Primary biliary cirrhosis

Peripheral nuclear

dsDNA

SLE

Nuclear matrix (coarse speckles)

hn-RNP

SLE

Coarse speckled

U1-RNP, Sm, Ki

SLE, MCTD

Fine speckled

Ro, La, Mi-2

SLE, Sjögren’s, dermatomysositis

Granular nuclear staining

Topoisomerase 1 (Scl-70)

Systemic sclerosis

Coarse and fine speckles

PCNA

SLE

Centromere (condensed in metaphase plates)

CENP-A, -B, -C

CREST, limited scleroderma

Nuclear dots (2–6) Nsp-1

p80 coilin

PBC

Nuclear dots (6–10, up to 30): pseudo-centromere

Sp100

PBC (AMA negative)

Homogeneous nucleolar

Pm-Scl, Ku, nucleolin

Polymyositis–scleroderma overlap

Clumpy nucleolar

Fibrillarin

Systemic sclerosis

Speckled nucleolar

RNA Pol 1

Systemic sclerosis

Nucleolar dots

Nor-90 (nucleolar organizer region)

Scleroderma

Centriole

Centriole

Non-specific (CTDs, post-infective)

Nuclear mitotic spindle apparatus (NuMa, MSA-1)

NuMA protein

Non-specific CTD

Mitotic spindle apparatus-2 (MSA-2)

Mid-body

Systemic sclerosis, Raynaud’s mid-body pattern

Mitotic spindle apparatus -3 (MSA-3)

CENP-F

Respiratory tract tumours

Cytoplasmic

Mitochondria (large granule staining)

Multiple antigens (M1–M9)

PBC, autoimmune liver disease

Ribosomes

Ribosomal P proteins

SLE

Irregular granular staining

Lysosomes, peroxisomes

Non-specific

Fine speckled

Jo-1, other tRNA synthetases

Polymyositis

Golgi body

Giantin (50%) golgins 245, 160, 95, 97

SLE, Sjögren’s, other CTDs, chronic CMV

Actin filaments

Actin

Autoimmune hepatitis

Vimentin

Vimentin

SLE, other CTDs

Cytokeratin

Cytokeratin 18

RhA

HEp-2 staining patternAntigen(s)Disease associations
Nuclear staining

Homogeneous nuclear/nucleolar

dsDNA, histones, topoisomerase 1, Ku

SLE

Homogeneous nuclear (nucleoli negative)

Histone

SLE, drug-induced SLE

Nuclear membrane

Lamins A, B, C

Autoimmune liver disease anti-phospholipid syndrome leucocystoclastic vasculitis

Nuclear pore (punctate staining)

gp210 pore protein, lamin B receptor

Primary biliary cirrhosis

Peripheral nuclear

dsDNA

SLE

Nuclear matrix (coarse speckles)

hn-RNP

SLE

Coarse speckled

U1-RNP, Sm, Ki

SLE, MCTD

Fine speckled

Ro, La, Mi-2

SLE, Sjögren’s, dermatomysositis

Granular nuclear staining

Topoisomerase 1 (Scl-70)

Systemic sclerosis

Coarse and fine speckles

PCNA

SLE

Centromere (condensed in metaphase plates)

CENP-A, -B, -C

CREST, limited scleroderma

Nuclear dots (2–6) Nsp-1

p80 coilin

PBC

Nuclear dots (6–10, up to 30): pseudo-centromere

Sp100

PBC (AMA negative)

Homogeneous nucleolar

Pm-Scl, Ku, nucleolin

Polymyositis–scleroderma overlap

Clumpy nucleolar

Fibrillarin

Systemic sclerosis

Speckled nucleolar

RNA Pol 1

Systemic sclerosis

Nucleolar dots

Nor-90 (nucleolar organizer region)

Scleroderma

Centriole

Centriole

Non-specific (CTDs, post-infective)

Nuclear mitotic spindle apparatus (NuMa, MSA-1)

NuMA protein

Non-specific CTD

Mitotic spindle apparatus-2 (MSA-2)

Mid-body

Systemic sclerosis, Raynaud’s mid-body pattern

Mitotic spindle apparatus -3 (MSA-3)

CENP-F

Respiratory tract tumours

Cytoplasmic

Mitochondria (large granule staining)

Multiple antigens (M1–M9)

PBC, autoimmune liver disease

Ribosomes

Ribosomal P proteins

SLE

Irregular granular staining

Lysosomes, peroxisomes

Non-specific

Fine speckled

Jo-1, other tRNA synthetases

Polymyositis

Golgi body

Giantin (50%) golgins 245, 160, 95, 97

SLE, Sjögren’s, other CTDs, chronic CMV

Actin filaments

Actin

Autoimmune hepatitis

Vimentin

Vimentin

SLE, other CTDs

Cytokeratin

Cytokeratin 18

RhA

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