It is difficult to say that there is more evidence for cardiac amyloidosis (CA) than for ischemic heart disease. On the other hand, 99 m technetium pyrophosphate ( 99 m Tc-PYP) scintigraphy has been reported to be useful with high sensitivity and specificity, especially in transthyretin (TTR) amyloidosis (ATTR) 1,2 Due to the spread of diagnosis using this method, CA, especially wild-type ATTR (ATTRwt) amyloidosis, which has traditionally been considered a rare disease, is more prevalent than previously assumed, and encountered relatively frequently in daily clinical practice. Furthermore, treatment for not only amyloid light-chain (AL) amyloidosis, but also ATTR, has also progressed rapidly. Tafamidis, a drug that stabilizes the TTR tetramer and suppresses amyloid fibril formation and tissue deposition, was listed and used in Japan in November 2013 for the treatment of peripheral neuropathy in patients with hereditary (variant) ATTR (ATTRv) amyloidosis. In addition, following the results of the 2018 Transthyretin Amyloidosis Cardiomyopathy Clinical Trial (ATTR-ACT), which showed the efficacy of tafamidis for CA, 3 the use of TTR to treat CA was approved in March 2019 in Japan.Needless to say, amyloidosis is a systemic disease, and in Japan, numerous studies have been conducted and medical treatments devised by the Ministry of Health, Labour and Welfare (MHLW)'s "Research Group on Amyloidosis", a research project on intractable disease policy. The present guidelines have been developed in coordination between the MHLW's "Research Group on Amyloidosis", the Japanese Circulation Society (JCS) and cardiology-related societies, the Japanese Society of Amyloidosis, and the Japanese Society of Hematology. Systemic amyloidosis specified by the MHLW as being an incurable disease is classified into the following four types.

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Pulmonary nosocomial infection.

Background and PurposeThe Japanese Clinical Practice Guidelines for Management of Sepsis and Septic Shock 2016 (J‐SSCG 2016), a Japanese‐specific set of clinical practice guidelines for sepsis and septic shock created jointly by the Japanese Society of Intensive Care Medicine and the Japanese Association for Acute Medicine, was first released in February 2017 in Japanese. An English‐language version of these guidelines was created based on the contents of the original Japanese‐language version.MethodsMembers of the Japanese Society of Intensive Care Medicine and the Japanese Association for Acute Medicine were selected and organized into 19 committee members and 52 working group members. The guidelines were prepared in accordance with the Medical Information Network Distribution Service (Minds) creation procedures. The Academic Guidelines Promotion Team was organized to oversee and provide academic support to the respective activities allocated to each Guideline Creation Team. To improve quality assurance and workflow transparency, a mutual peer review system was established, and discussions within each team were open to the public. Public comments were collected once after the initial formulation of a clinical question (CQ), and twice during the review of the final draft. Recommendations were determined to have been adopted after obtaining support from a two‐thirds (>66.6%) majority vote of each of the 19 committee members.ResultsA total of 87 CQs were selected among 19 clinical areas, including pediatric topics and several other important areas not covered in the first edition of the Japanese guidelines (J‐SSCG 2012). The approval rate obtained through committee voting, in addition to ratings of the strengths of the recommendation and its supporting evidence were also added to each recommendation statement. We conducted meta‐analyses for 29 CQs. Thirty seven CQs contained recommendations in the form of an expert consensus due to insufficient evidence. No recommendations were provided for 5 CQs.ConclusionsBased on the evidence gathered, we were able to formulate Japanese‐specific clinical practice guidelines that are tailored to the Japanese context in a highly transparent manner. These guidelines can easily be used not only by specialists, but also by non‐specialists, general clinicians, nurses, pharmacists, clinical engineers, and other healthcare professionals.

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