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Clinical and serolo...
Clinical and serologic parallels to APS-I in patients with thymomas and autoantigen transcripts in their tumors
- Article/chapterEnglish2014
Publisher, publication year, extent ...
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2014-10-15
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The American Association of Immunologists,2014
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LIBRIS-ID:oai:prod.swepub.kib.ki.se:130045512
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http://kipublications.ki.se/Default.aspx?queryparsed=id:130045512URI
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https://doi.org/10.4049/jimmunol.1401068DOI
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Language:English
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Summary in:English
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
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Patients with the autoimmune polyendocrine syndrome type I (APS-I), caused by mutations in the autoimmune regulator (AIRE) gene, and myasthenia gravis (MG) with thymoma, show intriguing but unexplained parallels. They include uncommon manifestations like autoimmune adrenal insufficiency (AI), hypoparathyroidism, and chronic mucocutaneous candidiasis plus autoantibodies neutralizing IL-17, IL-22, and type I IFNs. Thymopoiesis in the absence of AIRE is implicated in both syndromes. To test whether these parallels extend further, we screened 247 patients with MG, thymoma, or both for clinical features and organ-specific autoantibodies characteristic of APS-I patients, and we assayed 26 thymoma samples for transcripts for AIRE and 16 peripheral tissue-specific autoantigens (TSAgs) by quantitative PCR. We found APS-I–typical autoantibodies and clinical manifestations, including chronic mucocutaneous candidiasis, AI, and asplenia, respectively, in 49 of 121 (40%) and 10 of 121 (8%) thymoma patients, but clinical features seldom occurred together with the corresponding autoantibodies. Both were rare in other MG subgroups (n = 126). In 38 patients with APS-I, by contrast, we observed neither autoantibodies against muscle Ags nor any neuromuscular disorders. Whereas relative transcript levels for AIRE and 7 of 16 TSAgs showed the expected underexpression in thymomas, levels were increased for four of the five TSAgs most frequently targeted by these patients’ autoantibodies. Therefore, the clinical and serologic parallels to APS-I in patients with thymomas are not explained purely by deficient TSAg transcription in these aberrant AIRE-deficient tumors. We therefore propose additional explanations for the unusual autoimmune biases they provoke. Thymoma patients should be monitored for potentially life-threatening APS-I manifestations such as AI and hypoparathyroidism.
Added entries (persons, corporate bodies, meetings, titles ...)
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Karner, J
(author)
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Owe, JF
(author)
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Oftedal, BEV
(author)
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Gilhus, NE
(author)
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Erichsen, MM
(author)
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Kampe, OKarolinska Institutet
(author)
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Meager, A
(author)
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Peterson, P
(author)
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Kisand, K
(author)
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Willcox, N
(author)
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Husebye, ES
(author)
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Karolinska Institutet
(creator_code:org_t)
Related titles
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In:Journal of immunology (Baltimore, Md. : 1950): The American Association of Immunologists193:8, s. 3880-38901550-66060022-1767
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Wolff, ASB
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Karner, J
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Owe, JF
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Oftedal, BEV
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Gilhus, NE
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Erichsen, MM
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Kampe, O
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Meager, A
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Peterson, P
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Kisand, K
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Willcox, N
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Husebye, ES
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Journal of immun ...
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Karolinska Institutet