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.