2016 Guidelines for the Management of Thyroid Storm from the Japan
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2016, 63 (12), 1025-1064 OPINION 2016 Guidelines for the management of thyroid storm from The Japan Thyroid Association and Japan Endocrine Society (First edition) The Japan Thyroid Association and Japan Endocrine Society Taskforce Committee for the establishment of diagnostic criteria and nationwide surveys for thyroid storm Tetsurou Satoh1), Osamu Isozaki2), Atsushi Suzuki3), Shu Wakino4), Tadao Iburi5), Kumiko Tsuboi6), Naotetsu Kanamoto7) *, Hajime Otani8), Yasushi Furukawa9), Satoshi Teramukai10) and Takashi Akamizu9) 1) Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Gunma 371-8511, Japan 2) Department of Medicine 2, Tokyo Women’s Medical University, Tokyo 162-8666, Japan 3) Division of Endocrinology and Metabolism, Fujita Health University, Aichi 470-1192, Japan 4) Department of Endocrinology, Metabolism and Nephrology, Keio University, Tokyo 160-8582, Japan 5) Department of Endocrinology, Tenri Hospital, Nara 632-8552, Japan 6) Division of Diabetes, Metabolism, and Endocrinology, Department of Medicine, Toho University School of Medicine, Tokyo 143-8541, Japan 7) Kyoto University Graduate School of Medicine, Kyoto 606-8507, Japan 8) Department of Internal Medicine II, Kansai Medical University, Osaka 573-1010, Japan 9) The First Department of Medicine, Wakayama Medical University, Wakayama 641-8509, Japan 10) Department of Biostatistics, Kyoto Prefectural University of Medicine, Kyoto 602-8566, Japan Abstract. Thyroid storm is an endocrine emergency which is characterized by multiple organ failure due to severe thyrotoxicosis, often associated with triggering illnesses. Early suspicion, prompt diagnosis and intensive treatment will improve survival in thyroid storm patients. Because of its rarity and high mortality, prospective intervention studies for the treatment of thyroid storm are difficult to carry out. We, the Japan Thyroid Association and Japan Endocrine Society taskforce committee, previously developed new diagnostic criteria and conducted nationwide surveys for thyroid storm in Japan. Detailed analyses of clinical data from 356 patients revealed that the mortality in Japan was still high (~11%) and that multiple organ failure and acute heart failure were common causes of death. In addition, multimodal treatment with antithyroid drugs, inorganic iodide, corticosteroids and beta-adrenergic antagonists has been suggested to improve mortality of these patients. Based on the evidence obtained by nationwide surveys and additional literature searches, we herein established clinical guidelines for the management of thyroid storm. The present guideline includes 15 recommendations for the treatment of thyrotoxicosis and organ failure in the central nervous system, cardiovascular system, and hepato-gastrointestinal tract, admission criteria for the intensive care unit, and prognostic evaluation. We also proposed preventive approaches to thyroid storm, roles of definitive therapy, and future prospective trial plans for the treatment of thyroid storm. We hope that this guideline will be useful for many physicians all over the world as well as in Japan in the management of thyroid storm and the improvement of its outcome. Key words: Thyroid crisis, Diagnostic criteria, Prognosis assessment, Prevention, Thyrotoxicosis DISCLAIMER STATEMENT: These recommenda- of all proper approaches or methods, or exclusive of tions are developed to assist endocrinologists by pro- others. These recommendations cannot guarantee any viding guidance for regarding particular areas of prac- specific outcome and they do not establish a standard tice. The guidance should not be considered inclusive of care. The recommendations are not intended to dic- tate the treatment of any particular patient. Treatment Submitted Jul. 8, 2016; Accepted Sep. 2, 2016 as EJ16-0336 decisions must be made based on the independent judg- Released online in J-STAGE as advance publication Oct. 15, 2016 ment of healthcare providers and each patient’s indi- Correspondence to: Takashi Akamizu, M.D., Ph.D., The First vidual circumstances. Department of Medicine, Wakayama Medical University, Wakayama 641-8509, Japan. E-mail: [email protected] * N.K.’s current affiliation is Department of Endocrinology, Osaka The Japan Thyroid Association and the Japan City General Hospital, Osaka 534-0021, Japan. Endocrine Society make no warranty, express or ©The Japan Endocrine Society 1026 The JTA and JES Taskforce Committee for the establishment of diagnostic criteria and nationwide surveys for thyroid storm implied, regarding the guidance, and specifically heart failure, respiratory failure, arrhythmia, dissemi- exclude any warranties of merchantability and fitness nated intravascular coagulation (DIC), gastrointesti- for a particular use or purpose. The Japan Thyroid nal perforation, hypoxic brain syndrome, and sepsis. Association and the Japan Endocrine Society shall not Even when patients survive, some have irreversible be liable for direct, indirect, special, or consequen- damage including brain damage, disuse atrophy, cere- tial damages related to the use of the information con- brovascular disease, renal insufficiency, and psychosis. tained herein. Therefore, the prognosis of patients with thyroid storm needs to be improved. Table of Contents Since multiple organ failure is characteristic of thyroid storm, multidisciplinary expertise and care Introduction/Background involving endocrinologists, cardiologists, neurolo- gists, and hepatologists are necessary for manage- Diagnostic and therapeutic recommendations for ment. Furthermore, the decompensated state associ- thyroid storm ated with thyroid storm often requires comprehensive 1. Diagnostic challenges for thyroid storm and highly advanced medical treatment. Although sev- 2. Management of thyroid storm with antithy- eral textbooks and guidelines have described the treat- roid drugs, inorganic iodide, corticosteroids, ment of thyroid storm [3, 5-7], nationwide surveys in and antipyretics Japan revealed that methimazole (MMI) was preferen- 3. Use of therapeutic plasmapheresis to treat thy- tially used in thyroid storm despite recommendations roid storm for the use of propylthiouracil (PTU) [8]. Therefore, 4. Treatment of central nervous system manifes- the establishment of more detailed guidelines for the tations in thyroid storm management of thyroid storm is needed in Japan and 5. Treatment of tachycardia and atrial fibrillation other countries. Such guidelines should be helpful to in thyroid storm many practitioners. 6. Treatment of acute congestive heart failure in New diagnostic criteria for thyroid storm, in addi- thyroid storm tion to those of Burch and Wartofsky [3, 4, 9], have 7. Treatment of gastrointestinal disorders and been established. The next obvious step is to iden- hepatic damage in thyroid storm tify therapeutic procedures that improve prognosis 8. Recommended admission criteria for the [10, 11]. Five areas are important in the treatment intensive care unit and therapeutic strategy of thyroid storm: 1) thyrotoxicosis (reduction of thy- for comorbidities roid hormone secretion and production); 2) systemic 9. Prognostic evaluation of thyroid storm symptoms and signs (including high fever, dehydra- 10. Prevention of thyroid storm and roles of defin- tion, shock, and DIC); 3) organ-specific manifesta- itive treatment tions, such as cardiovascular, neurological, and hep- 11. An algorithm for the diagnosis and manage- ato-gastrointestinal; 4) triggers; and 5) definitive ment of thyroid storm therapy. Although the appropriate responses to these 12. Future directions for clinical trials in the man- problems have been described in endocrinology text- agement of thyroid storm books and reviews, several clinical questions remain, for example: 1) the choice and route of administration Introduction/Background for antithyroid drugs (ATDs), 2) timing of iodide ther- apy, 3) criteria to judge thyroid storm severity, and 4) Thyroid storm is a life-threatening condition that choice and fine-tuning of treatment based on severity requires rapid diagnosis and emergent treatment [1-3]. and pathophysiological state. Although beta-adrener- The condition manifests as decompensation of mul- gic receptor antagonists (beta-AAs) are often used to tiple organs with loss of consciousness, high fever, treat thyroid storm, inappropriate choice or dose may heart failure, diarrhea, and jaundice. Recent nation- lead to worse outcomes in patients with severe heart wide surveys in Japan have revealed that mortality failure [8]. Furthermore, thyroid storm is character- remains over 10% [4]. Multiple organ failure was the ized by multiple organ failure, decompensation, and most common cause of death, followed by congestive highly variable clinical presentation, a clinical pic- Guidelines of thyroid storm management 1027 ture that requires comprehensive treatment. Thyroid strong and quality of evidence is high or moder- storm is an emergent disorder characterized by rapid ate, the clinical practice can be applicable to most deterioration in its clinical course. Therefore, an patients in most circumstances without reservation. algorithm-based approach is useful for the manage- If the strength of recommendation is strong and ment of thyroid storm. quality of evidence is low, the recommendation may Given this context, we attempted to create recom- change when higher-quality evidence becomes avail- mendations for the management of thyroid storm based able. If the strength of recommendation is weak