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| Previous Nomenclature | None |
| Description | Diffuse fluorescence of the entire nucleolus, while the metaphase plate shows no staining. Frequently seen in combination with a fine speckled nuclear staining (AC-4) |
| Antigen Association | PM/Scl-75, PM/Scl-100, Th/To, B23/nucleophosmin, C23/nucleolin, No55/SC65 |
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Clinical Relevance
First level information About Clinical Relevance & List of Abbreviations |
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▶ Found in patients with systemic sclerosis (SSc), SSc-idiopathic inflammatory myopathies (IIM) overlap, and patients with clinical manifestations of other systemic autoimmune rheumatic diseases (SARD) [1, 2] ▶ If limited cutaneous SSc is clinically suspected, it is recommended to perform a follow-up test for Th/To antibodies (i.e., SSc profile) [1, 2] ▶ If SSc-IIM overlap syndrome is clinically suspected, it is recommended to perform a follow-up test for PM/Scl antibody reactivity; in general, PM/Scl antibodies yield a diffuse nuclear fine speckled staining in addition to the AC-8 pattern [3, 4] ▶ Other antigens recognized include B23/nucleophosmin, No55/SC65, and C23/nucleolin, but the clinical significance of these autoantibodies is not well established; specific immunoassays for these autoantibodies are currently not commercially available Th/To is a macromolecular complex comprising several autoantibody targets (hPOP1, Rpp25, Rpp38) with varying sensitivity and specificity for autoimmune disease. It is important to know which of these antigens are used in immunoassays [1, 5] |
| Second level information |
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▶ The AC‐8 pattern resulting from the anti‐Th/To reactivity is also seen in rare patients with systemic lupus erythematosus (SLE), undifferentiated connective tissue disease (UCTD; i.e., patients with rheumatic symptoms not satisfying a SARD diagnosis), SSc sine scleroderma, idiopathic interstitial lung disease and pulmonary hypertension [6, 7] ▶ Patients with autoantibodies revealing the AC‐8 pattern due to anti‐PM/Scl reactivity may have, in addition to the clinical features of IIM and SSc, various clinical manifestations of SLE and Sjögren’s disease [3] |
| References |
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1. Mahler M, Fritzler MJ, Satoh M. Autoantibodies to the mitochondrial RNA processing (MRP) complex also known as Th/To autoantigen. Autoimmun Rev. 2015;14:254-7 2. Ceribelli A, Cavazzana I, Franceschini F, Airo P, Tincani A, Cattaneo R, Pauley BA, Chan EKL, et al. Anti-Th/To are common antinucleolar autoantibodies in Italian patients with scleroderma. J Rheumatol. 2010;37:2071-5 3. Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev. 2007;6:432-7 4. D'Aoust J, Hudson M, Tatibouet S, Wick J, Canadian Scleroderma Research G, Mahler M, Baron M, Fritzler MJ. Clinical and serologic correlates of anti-PM/Scl antibodies in systemic sclerosis: a multicenter study of 763 patients. Arthritis Rheumatol. 2014;66:1608-15 5. Mahler M, Gascon C, Patel S, Ceribelli A, Fritzler MJ, Swart A, Chan EKL, Satoh M. Rpp25 is a major target of autoantibodies to the Th/To complex as measured by a novel chemiluminescent assay. Arthritis Res Ther. 2013;15:R50 6. Fischer A, Pfalzgraf FJ, Feghali-Bostwick CA, Wright TM, Curran-Everett D, West SG, Brown KK. Anti-th/to-positivity in a cohort of patients with idiopathic pulmonary fibrosis. J Rheumatol. 2006;33:1600-5 7. Fischer A, Meehan RT, Feghali-Bostwick CA, West SG, Brown KK. Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. Chest. 2006;130:976-81 |
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