{
  "abstract": "Since establishment in 2020 of the global consensus-2 diagnostic criteria for mast cell activation syndrome (MCAS), recognition of this prevalent disease, existing alongside rare cutaneous or systemic mastocytosis, has grown significantly. Despite this progress, some have continued using more restrictive criteria, significantly underdiagnosing this complex but treatable disorder. Consensus-2 has brought diagnosis and effective treatment in many MCAS patients previously labeled with unexplained (mostly inflammatory) syndromes or misdiagnosed as somatization or other primary psychiatric disorders. Fears that consensus-2 diagnostic criteria might bring overdiagnosis have not been realized. Appropriate therapy for MCAS can dramatically improve quality of life, sometimes after decades of morbidity and disability despite extensive past unhelpful workups and treatment. Appreciating the breadth and heterogeneity of MCAS, and achieving good management outcomes, requires understanding the complexity of mast cell biology and pathophysiology as well as the range of comorbidities the disease can drive and which can aggravate the disease. The spectrum of diseases thought possibly rooted in variants of MCAS (or to which MCAS is significantly contributing) continues expanding, calling for more research. New therapeutic options have emerged, and clinicians have gained better appreciation for use of existing treatments and mitigation of impediments to treatment. MCAS research remains in early stages, hampered by many factors including limited awareness of the disease and challenges in objective assessment of treatment response. This review examines developments in awareness, education, clinical care, and research since consensus-2 emerged, while discussing challenges and opportunities for accelerating progress.",
  "abstract_shown": true,
  "affiliations": [
    "Department of Mast Cell Studies , AIM Center for Personalized Medicine , Purchase , NY , USA",
    "Department of Neurology , Jacobs School of Medicine and Biomedical Sciences, University of Buffalo , Buffalo , NY , USA",
    "Department of Anesthesiology , Medical College of Wisconsin , Wausau , WI , USA",
    "Department of Integrative Medicine , AIM Center for Personalized Medicine , Purchase , NY , USA",
    "Heart of the Valley Pediatric Cardiology , Pleasanton , CA , USA",
    "Department of Family Practice , University of Texas Health Science Center at San Antonio , San Antonio , TX , USA",
    "Sydney Oncoplastic Surgery , Sydney , Australia",
    "Division of Pediatric Hematology , Rady Children Healthcare Orange County, University of California at Irvine, and Center for Inherited Blood Disorders , Orange , CA , USA",
    "Department of Internal Medicine , University of Colorado School of Medicine , Aurora , CO , USA",
    "Department of Medicine , Gastrointestinal Alliance , St. Louis , MO , USA",
    "Department of Medicine, Section of Allergy and Immunology , University of Southern California , Los Angeles , CA , USA",
    "Institute of Human Genetics, University Hospital of Bonn , Bonn , Germany"
  ],
  "article_type": "review",
  "arxiv": null,
  "author_total": 12,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Lawrence B. Afrin",
      "orcid": "0000-0003-3550-4244"
    },
    {
      "affs": [
        1
      ],
      "name": "Svetlana Blitshteyn",
      "orcid": "0000-0002-3982-2031"
    },
    {
      "affs": [
        2
      ],
      "name": "Linda S. Bluestein",
      "orcid": "0000-0002-5714-8714"
    },
    {
      "affs": [
        3
      ],
      "name": "Tania T. Dempsey",
      "orcid": "0000-0002-5325-0412"
    },
    {
      "affs": [
        4
      ],
      "name": "Andrew J. Maxwell",
      "orcid": "0000-0003-0781-4333"
    },
    {
      "affs": [
        5
      ],
      "name": "Claudia S. Miller"
    },
    {
      "affs": [
        6
      ],
      "name": "Èva Nagy"
    },
    {
      "affs": [
        7
      ],
      "name": "Diane J. Nugent"
    },
    {
      "affs": [
        8
      ],
      "name": "Jill R. Schofield",
      "orcid": "0000-0002-0053-3996"
    },
    {
      "affs": [
        9
      ],
      "name": "Leonard B. Weinstock",
      "orcid": "0000-0001-6484-1691"
    },
    {
      "affs": [
        10
      ],
      "name": "Shijun Cindy Xi"
    },
    {
      "affs": [
        11
      ],
      "name": "Gerhard Josef Molderings",
      "orcid": "0000-0002-9005-2749"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/bc12k4e8vvt7r.bib",
  "cited_by": 6,
  "doi": "10.1515/dx-2026-0016",
  "fulltext_url": null,
  "id": "bc12k4e8vvt7r",
  "issns": [
    "2194-8011",
    "2194-802X"
  ],
  "landing_url": "https://doi.org/10.1515/dx-2026-0016",
  "language": "en",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "licence_source": "journal",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "2403|Mast cells and histamine"
    ]
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  "openalex": "W7163506319",
  "pdf_url": null,
  "pmcid": null,
  "pmid": "42240520",
  "published": "2026-06-04",
  "publisher": "De Gruyter",
  "ris": "https://research.draconitic.com/paper/bc12k4e8vvt7r.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T13:26:51Z",
      "native_id": "W7163506319",
      "source": "openalex",
      "url": "https://doi.org/10.1515/dx-2026-0016"
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  "subjects": [
    {
      "field": "biology",
      "origin": "openalex:2403|Mast cells and histamine",
      "subfield": "immunology"
    }
  ],
  "title": "Progress in mast cell activation syndrome: the global consensus-2 diagnostic criteria at six years",
  "title_text": "Progress in mast cell activation syndrome: the global consensus-2 diagnostic criteria at six years",
  "type": "peer-reviewed",
  "updated_at": "2026-11-05T13:20:55Z",
  "url": "https://research.draconitic.com/paper/bc12k4e8vvt7r",
  "venue": "Diagnosis",
  "versions": []
}