Laboratory Medicine and Quality Assurance

Table. 1.

International consensus for nuclear, cytoplasmic, and mitotic patterns associated with autoimmune diseases [4-8]

Patterns Category Synonyms Related antigens Related diseases
Nuclear pattern Homogeneous (AC-1) Diffuse Double-stranded DNA, nucleosomes, histones SLE, drug-induced lupus, juvenile idiopathic arthritis
Speckled Granular hnRNP, U1RNP, Sm, SS-A/Ro (Ro60), SS-B/La, RNA polymerase III, Mi-2, Ku MCTD, SLE, SjS, DM, SSc/PM overlap
Dense fine speckled (AC-2) None DFS70/LEDGF Rare in SLE, SjS, SSc
Fine speckled (AC-4) Fine granular SS-A/Ro (Ro60), SS-B/La, Mi-2, TIF1γ, TIF1β, Ku, RNAhelicaseA, ReplicationproteinA SjS, SLE, DM, SSc/PM overlap
Large/coarse speckled (AC-5) Spliceosome/nuclear matrix hnRNP, U1RNP, Sm, RNA polymerase III MCTD, SLE, SSc
Centromere (AC-3) Kinetochore CENP-A/B (C) Limited cutaneous SSc, PBC
Multiple nuclear dots (AC-6) 6–20 nuclear dots, NSpI, PML bodies Sp100, PML proteins, MJ/NXP-2 PBC, SARD, PM/DM
Few nuclear dots (AC-7) 1–6 nuclear dots, Cajal bodies p80-coilin, SMN SjS, SLE, SSc, PM
Homogeneous nucleolar (AC-8) None PM/Scl-75, PM/Scl-100, Th/To, B23/nucleophosmin, nucleolin, No55/SC65 SSc, SSc/PM overlap
Clumpy nucleolar (AC-9) None U3-snoRNP/fibrillarin SSc
Punctate nucleolar (AC-10) Nucleolar speckled RNA polymerase I, hUBF/NOR-90 SSc, SjS
Smooth nuclear envelope (AC-11) Nuclear rim, nuclear membrane, membranous Lamins A, B, C, or lamin-associated proteins SLE, SjS, seronegative arthritis
Punctate nuclear envelope (AC-12) Nuclear membrane pores Nuclear pore complex proteins PBC
PCNA-like (AC-13) None PCNA SLE, other conditions
CENP-F-like (AC-14) MSA-3, NSp-II CENP-F Cancer, other conditions
Cytoplasmic pattern Linear/actin fibrillar (AC-15) Actin-like Actin, non-muscle myosin MCTD, chronic active hepatitis, liver cirrhosis, myasthenia gravis, Crohn’s disease, PBC, long-term hemodialysis, rare in SARD other than MCTD
Filamentous/microtubules (AC-16) None Vimentin, cytokeratins Infectious or inflammatory conditions, long-term hemodialysis, alcoholic liver disease, SARD, psoriasis, healthy controls
Segmental fibrillary (AC-17) None Alpha-actinin, vinculin, tropomyosin Myasthenia gravis, Crohn’s disease, ulcerative colitis
Discrete dots (AC-18) GW body, processing body, lysosome GW182, Su/Ago2, Ge-1 PBC, SARD, neurological and autoimmune conditions
Dense fine speckled (AC-19) Homogeneous PL-7, PL-12, ribosomal P proteins Anti-synthetase syndrome, PM/DM, SLE, juvenile SLE, neuropsychiatric SLE
Fine speckled (AC-20) Speckled Jo-1/histidyl-tRNA synthetase Anti-synthetase syndrome, PM/DM, limited SSc, idiopathic pleural effusion
Reticular/AMA (AC-21) Mitochondrion-like PDC-E2/M2, BCOADC-E2, OGDC-E2, E1α subunit of PDC, E3BP/proteinX Common in PBC, SSc, rare in other SARD
Polar/Golgi-like (AC-22) None Giantin/macrogolgin, golgin-95/GM130, golgin-160, golgin-97, golgin-245 Rare in SjS, SLE, RA, MCTD, GPA, idiopathic cerebellar ataxia, paraneoplastic cerebellar degeneration, viral infections
Rods and rings (AC-23) None IMPDH2, others HCV patients post-IFN/ribavirin therapy, rare in SLE, Hashimoto’s and healthy controls
Mitotic pattern Centrosome (AC-24) Centrioles Pericentrin, ninein, Cep250, Cep110, enolase Rare in SSc, Raynaud’s phenomenon, infections
Spindle fibers (AC-25) MSA-2 HsEg5 Rare in SjS, SLE, other SARD
NuMA-like (AC-26) MSA-1 Centrophilin SjS, SLE, other
Intercellular bridge (AC-27) Stem body, midbody Aurora kinase B, CENP-E, MSA-2, KIF-14, MKLP-1 Rare in SSc, Raynaud’s phenomenon, malignancy
Mitotic chromosome coat (AC-28) Chromosome coat protein, dividing cell antigen, MCA Modified histone H3, MCA-1 Rare in discoid lupus erythematosus, chronic lymphocytic leukemia, SjS, and polymyalgia rheumatica

Abbreviations: SLE, systemic lupus erythematosus; MCTD, mixed connective tissue disease; SjS, Sjögren’s syndrome; DM, dermatomyositis; SSc, systemic sclerosis; PM, polymyositis; PBC, primary biliary cirrhosis; SARD, systemic autoimmune rheumatic disease; AMA, anti-mitochondrial antibody; RA, rheumatoid arthritis; GPA, granulomatosis with polyangiitis; HCV, hepatitis C; IFN, interferon; NuMA, nuclear mitotic apparatus; MCA, mitotic chromosome autoantigen.

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