{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T08:54:57Z","timestamp":1789203297208,"version":"build-2803163510"},"reference-count":23,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2011,7,22]],"date-time":"2011-07-22T00:00:00Z","timestamp":1311292800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"},{"start":{"date-parts":[[2011,7,22]],"date-time":"2011-07-22T00:00:00Z","timestamp":1311292800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arthritis Res Ther"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                        <jats:title>Introduction<\/jats:title>\n                        <jats:p>Anti-RNA polymerase III (RNAP III) antibodies are highly specific markers of scleroderma (systemic sclerosis, SSc) and associated with a rapidly progressing subset of SSc. The clinical presentation of anti-RNAP III positive patients, onset of Raynaud's phenomenon (RP) and SSc in unselected patients in a rheumatology clinic were evaluated.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Methods<\/jats:title>\n                        <jats:p>Autoantibodies in sera from 1,966 unselected patients (including 434 systemic lupus erythematosus (SLE), 119 SSc, 85 polymyositis\/dermatomyositis (PM\/DM)) in a rheumatology clinic were screened by radioimmunoprecipitation. Anti-RNAP III positive sera were also tested by immunofluorescence antinuclear antibodies and anti-RNAP III ELISA. Medical records of anti-RNAP III positive patients were reviewed.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Results<\/jats:title>\n                        <jats:p>Among 21 anti-RNAP III positive patients, 16 met the American College of Rheumatology (ACR) SSc criteria at the initial visit but 5 did not; diagnoses were vasculitis, early polyarthritis, renal failure with RP, interstitial lung disease, and Sj\u00f6gren's syndrome. The first two patients developed rapidly progressive diffuse SSc. An additional case presented with diffuse scleroderma without RP and RP developed two years later. Anti-RNAP III antibodies in these 6 cases of atypical clinical presentation were compared with those in 15 cases of typical (SSc with RP) cases. Anti-RNAP III levels by ELISA were lower in the former group (<jats:italic>P<\/jats:italic> = 0.04 by Mann-Whitney test) and 3 of 6 were negative versus only 1 of 15 negative in the latter (<jats:italic>P<\/jats:italic> &lt; 0.05 by Fisher's exact test). Three cases of non-SSc anti-RNAP III positive patients had predominant reactivity with RNAP I with weak RNAP III reactivity and had a strong nucleolar staining. Three anti-RNAP III patients, who did not have RP at the initial visit, developed RP months later. Scleroderma developed prior to RP in 5 out of 16 (31%) in the anti-RNAP III group, but this was rare in patients with other autoantibodies. The interval between the onset of RP to scleroderma was short in anti-RNAP III positive patients.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Conclusions<\/jats:title>\n                        <jats:p>Anti-RNAP III antibodies are highly specific for SSc; however, a subset of anti-RNAP III positive patients do not present as typical SSc. The interval between RP and scleroderma in this group is short, and 31% of patients developed scleroderma prior to RP in this group. Anti-RNAP III positive patients may not present as typical SSc and detecting anti-RNAP III may have predictive value.<\/jats:p>\n                     <\/jats:sec>","DOI":"10.1186\/ar3422","type":"journal-article","created":{"date-parts":[[2011,7,23]],"date-time":"2011-07-23T06:17:54Z","timestamp":1311401874000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Atypical clinical presentation of a subset of patients with anti-RNA polymerase III - non-scleroderma cases associated with dominant RNA polymerase I reactivity and nucleolar staining"],"prefix":"10.1186","volume":"13","author":[{"given":"Angela","family":"Ceribelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malgorzata E","family":"Krzyszczak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven J","family":"Ross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason YF","family":"Chan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward KL","family":"Chan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rufus W","family":"Burlingame","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tyler T","family":"Webb","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R","family":"Bubb","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric S","family":"Sobel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Westley H","family":"Reeves","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minoru","family":"Satoh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2011,7,22]]},"reference":[{"key":"3187_CR1","doi-asserted-by":"publisher","first-page":"219","DOI":"10.3109\/s10165-009-0155-3","volume":"19","author":"M Satoh","year":"2009","unstructured":"Satoh M, Vazquez-Del Mercado M, Chan EK: Clinical interpretation of antinuclear antibody tests in systemic rheumatic diseases. Mod Rheumatol. 2009, 19: 219-228. 10.1007\/s10165-009-0155-3.","journal-title":"Mod Rheumatol"},{"key":"3187_CR2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.rdc.2007.12.001","volume":"34","author":"VD Steen","year":"2008","unstructured":"Steen VD: The many faces of scleroderma. Rheum Dis Clin North Am. 2008, 34: 1-15. 10.1016\/j.rdc.2007.12.001.","journal-title":"Rheum Dis Clin North Am"},{"key":"3187_CR3","doi-asserted-by":"publisher","first-page":"1526","DOI":"10.1056\/NEJMoa021933","volume":"349","author":"MR Arbuckle","year":"2003","unstructured":"Arbuckle MR, McClain MT, Rubertone MV, Scofield RH, Dennis GJ, James JA, Harley JB: Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med. 2003, 349: 1526-1533. 10.1056\/NEJMoa021933.","journal-title":"N Engl J Med"},{"key":"3187_CR4","doi-asserted-by":"publisher","first-page":"1981","DOI":"10.1172\/JCI117550","volume":"94","author":"M Satoh","year":"1994","unstructured":"Satoh M, Ajmani AK, Ogasawara T, Langdon JJ, Hirakata M, Wang J, Reeves WH: Autoantibodies to RNA polymerase II are common in systemic lupus erythematosus and overlap syndrome. Specific recognition of the phosphorylated (IIO) form by a subset of human sera. J Clin Invest. 1994, 94: 1981-1989. 10.1172\/JCI117550.","journal-title":"J Clin Invest"},{"key":"3187_CR5","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1016\/j.semarthrit.2005.03.005","volume":"35","author":"VD Steen","year":"2005","unstructured":"Steen VD: Autoantibodies in systemic sclerosis. Semin Arthritis Rheum. 2005, 35: 35-42. 10.1016\/j.semarthrit.2005.03.005.","journal-title":"Semin Arthritis Rheum"},{"key":"3187_CR6","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1093\/jb\/mvq113","volume":"149","author":"HI Suzuki","year":"2011","unstructured":"Suzuki HI, Miyazono K: Emerging complexity of microRNA generation cascades. J Biochem. 2011, 149: 15-25. 10.1093\/jb\/mvq113.","journal-title":"J Biochem"},{"key":"3187_CR7","first-page":"1497","volume-title":"Arthritis and Allied Conditions. A Textbook of Rheumatology","author":"WH Reeves","year":"2005","unstructured":"Reeves WH, Narain S, Satoh M: Autoantibodies in systemic lupus erythematosus. Arthritis and Allied Conditions. A Textbook of Rheumatology. Edited by: Koopman WJ, Moreland LW. 2005, Philadelphia, PA: Lippincott Williams and Wilkins, 1497-1521. 15","edition":"15"},{"key":"3187_CR8","doi-asserted-by":"publisher","first-page":"R111","DOI":"10.1186\/ar2000","volume":"8","author":"Y Yamasaki","year":"2006","unstructured":"Yamasaki Y, Narain S, Hernandez L, Barker T, Ikeda K, Segal MS, Richards HB, Chan EK, Reeves WH, Satoh M: Autoantibodies against the replication protein A complex in systemic lupus erythematosus and other autoimmune diseases. Arthritis Res Ther. 2006, 8: R111-120. 10.1186\/ar2000.","journal-title":"Arthritis Res Ther"},{"key":"3187_CR9","doi-asserted-by":"publisher","first-page":"2665","DOI":"10.1172\/JCI116505","volume":"91","author":"M Hirakata","year":"1993","unstructured":"Hirakata M, Okano Y, Pati U, Suwa A, Medsger TAJ, Hardin JA, Craft J: Identification of autoantibodies to RNA polymerase II. Occurrence in systemic sclerosis and association with autoantibodies to RNA polymerases I and III. J Clin Invest. 1993, 91: 2665-2772. 10.1172\/JCI116505.","journal-title":"J Clin Invest"},{"key":"3187_CR10","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1006\/excr.1999.4739","volume":"254","author":"E Jones","year":"2000","unstructured":"Jones E, Kimura H, Vigneron M, Wang Z, Roeder RG, Cook PR: Isolation and characterization of monoclonal antibodies directed against subunits of human RNA polymerases I, II, and III. Exp Cell Res. 2000, 254: 163-172. 10.1006\/excr.1999.4739.","journal-title":"Exp Cell Res"},{"key":"3187_CR11","doi-asserted-by":"publisher","first-page":"444","DOI":"10.1002\/1529-0131(200002)43:2<444::AID-ANR27>3.0.CO;2-G","volume":"43","author":"H Poormoghim","year":"2000","unstructured":"Poormoghim H, Lucas M, Fertig N, Medsger TA: Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. Arthritis Rheum. 2000, 43: 444-451. 10.1002\/1529-0131(200002)43:2<444::AID-ANR27>3.0.CO;2-G.","journal-title":"Arthritis Rheum"},{"key":"3187_CR12","doi-asserted-by":"publisher","first-page":"976","DOI":"10.1378\/chest.130.4.976","volume":"130","author":"A Fischer","year":"2006","unstructured":"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: 976-981. 10.1378\/chest.130.4.976.","journal-title":"Chest"},{"key":"3187_CR13","first-page":"2489","volume":"26","author":"TG Phan","year":"1999","unstructured":"Phan TG, Cass A, Gillin A, Trew P, Fertig N, Sturgess A: Anti-RNA polymerase III antibodies in the diagnosis of scleroderma renal crisis sine scleroderma. J Rheumatol. 1999, 26: 2489-2492.","journal-title":"J Rheumatol"},{"key":"3187_CR14","doi-asserted-by":"publisher","first-page":"119","DOI":"10.1159\/000046327","volume":"90","author":"JJ Canet","year":"2002","unstructured":"Canet JJ, Castane J, Alvarez M, Nava JM, Llibre J: Scleroderma renal crisis sine scleroderma. Nephron. 2002, 90: 119-120. 10.1159\/000046327.","journal-title":"Nephron"},{"key":"3187_CR15","doi-asserted-by":"publisher","first-page":"2425","DOI":"10.1002\/art.21232","volume":"52","author":"M Kuwana","year":"2005","unstructured":"Kuwana M, Okano Y, Pandey JP, Silver RM, Fertig N, Medsger TA: Enzyme-linked immunosorbent assay for detection of anti-RNA polymerase III antibody: analytical accuracy and clinical associations in systemic sclerosis. Arthritis Rheum. 2005, 52: 2425-2432. 10.1002\/art.21232.","journal-title":"Arthritis Rheum"},{"key":"3187_CR16","doi-asserted-by":"publisher","first-page":"1570","DOI":"10.1093\/rheumatology\/kep290","volume":"48","author":"T Satoh","year":"2009","unstructured":"Satoh T, Ishikawa O, Ihn H, Endo H, Kawaguchi Y, Sasaki T, Goto D, Takahashi K, Takahashi H, Misaki Y, Mimori T, Muro Y, Yazawa N, Sato S, Takehara K, Kuwana M: Clinical usefulness of anti-RNA polymerase III antibody measurement by enzyme-linked immunosorbent assay. Rheumatology (Oxford). 2009, 48: 1570-1574. 10.1093\/rheumatology\/kep290.","journal-title":"Rheumatology (Oxford)"},{"key":"3187_CR17","doi-asserted-by":"publisher","first-page":"976","DOI":"10.1093\/rheumatology\/ken201","volume":"47","author":"JC Parker","year":"2008","unstructured":"Parker JC, Burlingame RW, Webb TT, Bunn CC: Anti-RNA polymerase III antibodies in patients with systemic sclerosis detected by indirect immunofluorescence and ELISA. Rheumatology (Oxford). 2008, 47: 976-979. 10.1093\/rheumatology\/ken201.","journal-title":"Rheumatology (Oxford)"},{"key":"3187_CR18","doi-asserted-by":"publisher","first-page":"3051","DOI":"10.1002\/art.22043","volume":"54","author":"Y Yamasaki","year":"2006","unstructured":"Yamasaki Y, Honkanen-Scott M, Hernandez L, Ikeda K, Barker T, Bubb MR, Narain S, Richards HB, Chan EK, Reeves WH, Satoh M: Nucleolar staining cannot be used as a screening test for the scleroderma marker anti-RNA polymerase I\/III antibodies. Arthritis Rheum. 2006, 54: 3051-3056. 10.1002\/art.22043.","journal-title":"Arthritis Rheum"},{"key":"3187_CR19","doi-asserted-by":"publisher","first-page":"1218","DOI":"10.1093\/rheumatology\/kep215","volume":"48","author":"SI Nihtyanova","year":"2009","unstructured":"Nihtyanova SI, Parker JC, Black CM, Bunn CC, Denton CP: A longitudinal study of anti-RNA polymerase III antibody levels in systemic sclerosis. Rheumatology (Oxford). 2009, 48: 1218-1221. 10.1093\/rheumatology\/kep215.","journal-title":"Rheumatology (Oxford)"},{"key":"3187_CR20","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1016\/j.autrev.2009.02.002","volume":"8","author":"I Cavazzana","year":"2009","unstructured":"Cavazzana I, Angela C, Paolo A, Stefania Z, Angela T, Franco F: Anti-RNA polymerase III antibodies: a marker of systemic sclerosis with rapid onset and skin thickening progression. Autoimmun Rev. 2009, 8: 580-584. 10.1016\/j.autrev.2009.02.002.","journal-title":"Autoimmun Rev"},{"key":"3187_CR21","doi-asserted-by":"publisher","first-page":"1228","DOI":"10.1046\/j.1365-2133.2000.03579.x","volume":"142","author":"C Querfeld","year":"2000","unstructured":"Querfeld C, Sollberg S, Huerkamp C, Eckes B, Krieg T: Pseudoscleroderma associated with lung cancer: correlation of collagen type I and connective tissue growth factor gene expression. Br J Dermatol. 2000, 142: 1228-1233. 10.1046\/j.1365-2133.2000.03579.x.","journal-title":"Br J Dermatol"},{"key":"3187_CR22","doi-asserted-by":"publisher","first-page":"1766","DOI":"10.1002\/art.11018","volume":"48","author":"T Fujii","year":"2003","unstructured":"Fujii T, Mimori T, Kimura N, Satoh S, Hirakata M: Pseudoscleroderma associated with transforming growth factor beta1-producing advanced gastric carcinoma: comment on the article by Varga. Arthritis Rheum. 2003, 48: 1766-1767; author repy 1767-1768. 10.1002\/art.11018.","journal-title":"Arthritis Rheum"},{"key":"3187_CR23","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1111\/j.1365-2230.2006.02092.x","volume":"31","author":"K Kikuchi","year":"2006","unstructured":"Kikuchi K, Hoashi T, Yazawa N, Tamaki K: Pseudoscleroderma associated with cancer. Clin Exp Dermatol. 2006, 31: 381-383. 10.1111\/j.1365-2230.2006.02092.x.","journal-title":"Clin Exp Dermatol"}],"container-title":["Arthritis Research &amp; Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/ar3422.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/ar3422\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/ar3422.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,8]],"date-time":"2024-10-08T11:06:48Z","timestamp":1728385608000},"score":1,"resource":{"primary":{"URL":"https:\/\/arthritis-research.biomedcentral.com\/articles\/10.1186\/ar3422"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,7,22]]},"references-count":23,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2011,8]]}},"alternative-id":["3187"],"URL":"https:\/\/doi.org\/10.1186\/ar3422","relation":{},"ISSN":["1478-6354"],"issn-type":[{"value":"1478-6354","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,7,22]]},"assertion":[{"value":"3 March 2011","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 May 2011","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 July 2011","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 July 2011","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"R119"}}