{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T09:09:35Z","timestamp":1790672975183,"version":"4.1.0"},"reference-count":28,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2018,3,1]],"date-time":"2018-03-01T00:00:00Z","timestamp":1519862400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Allergy"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Mastocytosis is a heterogeneous disease characterized by a clonal expansion of mast cells in various organs. The vast majority of patients suffer from signs and symptoms caused by mediator release from mast cells. Although the disease burden is high, there is currently no specific and validated instrument to measure and monitor signs and symptoms in patients with mastocytosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To develop and validate a disease\u2010specific tool to measure and monitor the activity of signs and symptoms in patients with mastocytosis, the Mastocytosis Activity Score (MAS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Nineteen potential <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> items were developed in a combined approach consisting of semi\u2010structured patient interviews, expert input and literature research. Item selection was performed by impact analysis with 76 patients followed by a review for face validity. The resulting <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> was tested for validity, reliability and influence factors. In parallel, a <jats:styled-content style=\"fixed-case\">US<\/jats:styled-content> American English version of the <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> was developed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 68 mastocytosis patients took part in the <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> validation study. The final 9\u2010item <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> was found to have a three\u2010domain structure (\u201cskin,\u201d \u201cgastrointestinal tract\u201d and \u201cother\u201d), a valid total score and an excellent test\u2010retest reliability. Multiple regression analysis revealed that disease duration, age or gender is not a significant determinant of the <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> results.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The <jats:styled-content style=\"fixed-case\">MAS<\/jats:styled-content> is a disease\u2010specific, valid and reliable patient\u2010reported outcome measure for adult patients with cutaneous and indolent systemic mastocytosis. It may serve as a valuable tool to measure and monitor mastocytosis activity, both, in clinical trials and in routine care.<\/jats:p><\/jats:sec>","DOI":"10.1111\/all.13425","type":"journal-article","created":{"date-parts":[[2018,2,6]],"date-time":"2018-02-06T06:45:16Z","timestamp":1517899516000},"page":"1489-1496","source":"Crossref","is-referenced-by-count":29,"title":["Development and validation of the mastocytosis activity score"],"prefix":"10.1111","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4532-1644","authenticated-orcid":false,"given":"F.","family":"Siebenhaar","sequence":"first","affiliation":[{"name":"Interdisciplinary Mastocytosis Center Charit\u00e9 Department of Dermatology and Allergy Charit\u00e9 \u2010 Universit\u00e4tsmedizin Berlin Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Sander","sequence":"additional","affiliation":[{"name":"Interdisciplinary Mastocytosis Center Charit\u00e9 Department of Dermatology and Allergy Charit\u00e9 \u2010 Universit\u00e4tsmedizin Berlin Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. 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