Diagnostic Criteria, Clinical Features, and Incidence of Thyroid Storm Based on Nationwide Surveys
Open accessAbstract
Background: Thyroid storm (TS) is life threatening. Its incidence is poorly defined, few series are available, and population-based diagnostic criteria have not been established. We surveyed TS in Japan, defined its characteristics, and formulated diagnostic criteria, FINAL-CRITERIA1 and FINAL-CRITERIA2, for two grades of TS, TS1, and TS2 respectively. Methods: We first developed diagnostic criteria based on 99 patients in the literature and 7 of our patients (LIT-CRITERIA1 for TS1 and LIT-CRITERIA2 for TS2). Thyrotoxicosis was a prerequisite for TS1 and TS2 as well as for combinations of the central nervous system manifestations, fever, tachycardia, congestive heart failure (CHF), and gastrointestinal (GI)/hepatic disturbances. We then conducted initial and follow-up surveys from 2004 through 2008, targeting all hospitals in Japan, with an eight-layered random extraction selection process to obtain and verify information on patients who met LIT-CRITERIA1 and LIT-CRITERIA2. Results: We identified 282 patients with TS1 and 74 patients with TS2. Based on these data and information from the Ministry of Health, Labor, and Welfare of Japan, we estimated the incidence of TS in hospitalized patients in Japan to be 0.20 per 100,000 per year. Serum-free thyroxine and free triiodothyroine concentrations were similar among patients with TS in the literature, Japanese patients with TS1 or TS2, and a group of patients with thyrotoxicosis without TS (Tox-NoTS). The mortality rate was 11.0% in TS1, 9.5% in TS2, and 0% in Tox-NoTS patients. Multiple organ failure was the most common cause of death in TS1 and TS2, followed by CHF, respiratory failure, arrhythmia, disseminated intravascular coagulation, GI perforation, hypoxic brain syndrome, and sepsis. Glasgow Coma Scale results and blood urea nitrogen (BUN) were associated with irreversible damages in 22 survivors. The only change in our final diagnostic criteria for TS as compared with our initial criteria related to serum bilirubin concentration >3 mg/dL. Conclusions: TS is still a life-threatening disorder with more than 10% mortality in Japan. We present newly formulated diagnostic criteria for TS and clarify its clinical features, prognosis, and incidence based on nationwide surveys in Japan. This information will help diagnose TS and in understanding the factors contributing to mortality and irreversible complications.
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Thyroid storm occurs only rarely following administration of RAI.
“Thyroid storm occurs only rarely following the administration of RAI (70)(71)(72).”
From 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis (2016) · cites this paper as [72]
Diagnostic criteria for thyroid storm in patients with severe thyrotoxicosis were first proposed in 1993 and subsequently widely adopted as the Burch-Wartofsky Point Scale (BWPS) for thyroid storm.
“Diagnostic criteria for thyroid storm in patients with severe thyrotoxicosis were first proposed in 1993 and subsequently widely adopted as the BWPS for thyroid storm (26,72,186,226,228).”
From 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis (2016) · cites this paper as [72]
Data comparing the BWPS and JTA diagnostic systems suggest an overall agreement, but a tendency toward underdiagnosis using the JTA categories of TS1 and TS2 compared to a BWPS ≥45.
“Data comparing these two diagnostic systems suggest an overall agreement, but a tendency toward underdiagnosis using the JTA categories of TS1 and TS2, compared to a BWPS ‡45 (72,186,226,227).”
From 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis (2016) · cites this paper as [72]
Precipitants of thyroid storm in a patient with previously compensated thyrotoxicosis include abrupt cessation of ATDs, thyroidectomy, or nonthyroidal surgery in a patient with unrecognized or inadequately treated thyrotoxicosis, and a number of acute illnesses unrelated to thyroid disease.
“Precipitants of thyroid storm in a patient with previously compensated thyrotoxicosis include abrupt cessation of ATDs, thyroidectomy, or nonthyroidal surgery in a patient with unrecognized or inadequately treated thyrotoxicosis, and a number of acute illnesses unrelated to thyroid disease (72,186,228).”
From 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis (2016) · cites this paper as [72]
The Japanese Thyroid Association (JTA) has proposed an additional empirically defined diagnostic system that uses combinations of similar clinical features to assign patients to diagnostic categories TS1 or TS2.
“Recently, an additional empirically defined diagnostic system has been proposed by the JTA (72).The JTA system uses combinations of similar clinical features to assign patients to the diagnostic categories TS1 or TS2.”
From 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis (2016) · cites this paper as [72]
A BWPS ≥45 appears more sensitive than a JTA classification of TS1 or TS2 in detecting patients with a clinical diagnosis of thyroid storm, but patients with a BWPS of 25-44 represent a group in whom the decision to use aggressive therapy should be based on sound clinical judgment and not based solely on diagnostic category in order to avoid overtreatment and the resultant risk of drug toxicity.
“A BWPS ‡45 appears more sensitive than a JTA classification of TS1 or TS2 in detecting patients with a clinical diagnosis of thyroid storm, but patients with a BWPS of 25-44 represent a group in whom the decision to use aggressive therapy should be based on sound clinical judgment and not based solely on diagnostic category in order to avoid overtreatment and the resultant risk of drug toxicity.”
From 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis (2016) · cites this paper as [72]
Life-threatening thyrotoxicosis or thyroid storm is a rare disorder characterized by multisystem involvement and mortality rates in the range of 8%-25% in modern series.
“Life-threatening thyrotoxicosis or thyroid storm is a rare disorder characterized by multisystem involvement and mortality rates in the range of 8%-25% in modern series (25,72,226,227).”
From 2016 American Thyroid Association Guidelines for Diagnosis and Management of Hyperthyroidism and Other Causes of Thyrotoxicosis (2016) · cites this paper as [72]
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- Authors
- Akamizu, T., Satoh, T., Isozaki, O., Suzuki, A., Wakino, S., Iburi, T., Tsuboi, K., Monden, T., Kouki, T., Otani, H., Teramukai, S., Uehara, R., Nakamura, Y., Nagai, M., & Mori, F. T. J. T. A.
- Affiliations
- Wakayama Medical University (JP)
- Gunma University (JP)
- Tokyo Women's Medical University (JP)
- Fujita Health University (JP)
- Keio University (JP)
- Tokyo Medical and Dental University (JP)
- Toho University (JP)
- Dokkyo Medical University (JP)
- University of the Ryukyus (JP)
- Kansai Medical University (JP)
- Kyoto University (JP)
- Jichi Medical University (JP)
- Saitama Medical University (JP)
- Gunma University
- Corresponding author
- Akamizu, Takashi
- ORCID
- Venue
- Thyroid®
- Publisher
- SAGE Publications
- Published
- 2012-07
- Volume
- 22
- Issue
- 7
- Pages
- 661-679
- ISSN
- 1050-7256 (ISSN-L)
- 1557-9077
- PMC
- PMC3387770
- OpenAlex
- W2013904710
- Cited by
- 469
- References
- 22
- Citations by year
- 2026: 32 · 2025: 43 · 2024: 53 · 2023: 33 · 2022: 42 · 2021: 43 · 2020: 36 · 2019: 32 · 2018: 30 · 2017: 21 · 2016: 30 · 2015: 21 · 2014: 32 · 2013: 12 · 2012: 8
- FWCI
- 18.73
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Thyroid (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Japan Society for the Promotion of Science (23591332, 24390204)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Thyroid Disorders and Treatments
- Health Sciences › Medicine › Pathology and Forensic Medicine › Ophthalmology and Eye Disorders
- Health Sciences › Medicine › Surgery › Thyroid and Parathyroid Surgery
- Keywords
- thyroid storm, diagnostic criteria, incidence, mortality, thyrotoxicosis, multiple organ failure, congestive heart failure, serum bilirubin, Glasgow Coma Scale, blood urea nitrogen, hospitalized patients, Japan, prognosis, clinical features, sepsis, cardiac arrhythmias, respiratory failure, disseminated intravascular coagulation, gastrointestinal perforation
- MeSH terms
- Adolescent, Adult, Aged, Aged, 80 and over, Blood Urea Nitrogen, Child, Disseminated Intravascular Coagulation, Female, Heart Failure, Humans, Japan, Male, Middle Aged, Multiple Organ Failure, Prognosis, Thyroid Crisis, Precipitating Factors, Incidence, Logistic Models, Case-Control Studies
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