Practice guideline for lipodystrophy syndromes—clinically important diseases of the Japan Endocrine Society (JES)

Lipodystrophy syndromes are a group of metabolic disorders that are either congenital or acquired in onset and are either generalized, partial, or regional in fat loss distribution. They frequently present lipodystrophy-related metabolic derangements or diseases such as marked insulin resistance, diabetes mellitus, hypertriglyceridemia, and nonalcoholic fatty liver disease (NAFLD). Severities are in parallel with the progress of lipodystrophy without correlation with energy intake. No essential therapy has been established to date. However, leptin replacement therapy was approved in 2013, first in Japan and then all over the world, based on its effectiveness and safety in lipodystrophy-related metabolic diseases. Moreover, the Japan Endocrine Society was put in charge of the data on lipodystrophy syndromes by the