{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T23:22:47Z","timestamp":1788477767095,"version":"build-2803163510"},"reference-count":27,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2022,4,7]],"date-time":"2022-04-07T00:00:00Z","timestamp":1649289600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Allergy"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Idiopathic mast cell activation syndrome (MCAS) is characterized by three diagnostic criteria: (1) episodic mast cell (MC)\u2010driven signs\/symptoms of at least two organ systems in the absence of clonal MC expansion and definite triggers, (2) episodic increase in tryptase, and (3) response to MC\u2010targeted treatment. Many patients believe they have MCAS, but how often this is the case remains unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We prospectively investigated patients with suspected MCAS (<jats:italic>n<\/jats:italic>\u00a0=\u00a0100) for the diagnostic criteria including baseline tryptase, KIT D816V mutation, and patient\u2010reported outcome measures (PROMs) over the course of 12\u00a0weeks. Comorbid depression and anxiety were explored with the Hospital Anxiety and Depression Scale (HADS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In 53% of our patients (80% females), suspicion of MCAS was based on self\u2010evaluation. In total, patients reported 87 different symptoms, mostly fatigue (<jats:italic>n<\/jats:italic>\u00a0=\u00a057), musculoskeletal pain\/weakness (<jats:italic>n<\/jats:italic>\u00a0=\u00a049), and abdominal pain (<jats:italic>n<\/jats:italic>\u00a0=\u00a043), with overall high disease activity and impact. Two of 79 patients had increased tryptase (by &gt;20% +2\u00a0ng\/ml) following an episode. Only 5%, with any of the PROMs used, showed complete response to MC\u2010targeted treatment. Depression and anxiety disorders were frequent comorbidities (<jats:italic>n<\/jats:italic>\u00a0=\u00a023 each), and 65 patients had pathological HADS values, which were linked to high disease impact and poor symptom control.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Mast cell activation syndrome was confirmed in only 2% of patients, which implies that it is not MC activation that drives signs and symptoms in most patients with suspected MCAS. There is a high need for comprehensive research efforts aimed at the identification of the true underlying pathomechanism(s) in patients with suspected MCAS.<\/jats:p><\/jats:sec>","DOI":"10.1111\/all.15304","type":"journal-article","created":{"date-parts":[[2022,4,1]],"date-time":"2022-04-01T21:08:47Z","timestamp":1648847327000},"page":"2794-2802","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Idiopathic mast cell activation syndrome is more often suspected than diagnosed\u2014A prospective real\u2010life study"],"prefix":"10.1111","volume":"77","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9389-1767","authenticated-orcid":false,"given":"Thomas","family":"Buttgereit","sequence":"first","affiliation":[{"name":"Institute of Allergology Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin Corporate Member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin  Berlin Germany"},{"name":"Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Allergology and Immunology  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Gu","sequence":"additional","affiliation":[{"name":"Institute of Allergology Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin Corporate Member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7032-4260","authenticated-orcid":false,"given":"Leonor","family":"Carneiro\u2010Le\u00e3o","sequence":"additional","affiliation":[{"name":"Allergy and Clinical Immunology Department Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, E.P.E.  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