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Peer-reviewedCC BYDiagnosis

Progress in mast cell activation syndrome: the global consensus-2 diagnostic criteria at six years

  • Lawrence B. Afrin1
  • Svetlana Blitshteyn2
  • Linda S. Bluestein3
  • Tania T. Dempsey4
  • Andrew J. Maxwell5
  • Claudia S. Miller6
  • Èva Nagy7
  • Diane J. Nugent8
  • Jill R. Schofield9
  • Leonard B. Weinstock10
  • Shijun Cindy Xi11
  • Gerhard Josef Molderings12
12 affiliations
  1. 1Department of Mast Cell Studies , AIM Center for Personalized Medicine , Purchase , NY , USA
  2. 2Department of Neurology , Jacobs School of Medicine and Biomedical Sciences, University of Buffalo , Buffalo , NY , USA
  3. 3Department of Anesthesiology , Medical College of Wisconsin , Wausau , WI , USA
  4. 4Department of Integrative Medicine , AIM Center for Personalized Medicine , Purchase , NY , USA
  5. 5Heart of the Valley Pediatric Cardiology , Pleasanton , CA , USA
  6. 6Department of Family Practice , University of Texas Health Science Center at San Antonio , San Antonio , TX , USA
  7. 7Sydney Oncoplastic Surgery , Sydney , Australia
  8. 8Division of Pediatric Hematology , Rady Children Healthcare Orange County, University of California at Irvine, and Center for Inherited Blood Disorders , Orange , CA , USA
  9. 9Department of Internal Medicine , University of Colorado School of Medicine , Aurora , CO , USA
  10. 10Department of Medicine , Gastrointestinal Alliance , St. Louis , MO , USA
  11. 11Department of Medicine, Section of Allergy and Immunology , University of Southern California , Los Angeles , CA , USA
  12. 12Institute of Human Genetics, University Hospital of Bonn , Bonn , Germany
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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.

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Cite

BibTeXRIS
BibTeX
@article{afrin2026progress,
  title = {{Progress in mast cell activation syndrome: the global consensus-2 diagnostic criteria at six years}},
  author = {Lawrence B. Afrin and Svetlana Blitshteyn and Linda S. Bluestein and Tania T. Dempsey and Andrew J. Maxwell and Claudia S. Miller and Èva Nagy and Diane J. Nugent and Jill R. Schofield and Leonard B. Weinstock and Shijun Cindy Xi and Gerhard Josef Molderings},
  journal = {Diagnosis},
  year = {2026},
  month = jun,
  doi = {10.1515/dx-2026-0016},
  url = {https://doi.org/10.1515/dx-2026-0016},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - Progress in mast cell activation syndrome: the global consensus-2 diagnostic criteria at six years
AU  - Lawrence B. Afrin
AU  - Svetlana Blitshteyn
AU  - Linda S. Bluestein
AU  - Tania T. Dempsey
AU  - Andrew J. Maxwell
AU  - Claudia S. Miller
AU  - Èva Nagy
AU  - Diane J. Nugent
AU  - Jill R. Schofield
AU  - Leonard B. Weinstock
AU  - Shijun Cindy Xi
AU  - Gerhard Josef Molderings
PY  - 2026
DA  - 2026/06/04
JO  - Diagnosis
DO  - 10.1515/dx-2026-0016
UR  - https://doi.org/10.1515/dx-2026-0016
AB  - 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.
SN  - 2194-8011
SN  - 2194-802X
ER  -