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Treatment of erythema nodosum leprosum (ENL, type 2 lepra reaction) with thalidomide is an effective alternative to steroid therapy, but Ministry of Health, Labour and Welfare (Japan) has only approved thalidomide (Thaled Capsule, Fujimoto Pharmaceutical Corp.) for the treatment of multiple myeloma under the Thalidomide Education and Risk Management System (TERMS) 2008. Then thalidomide was approved for ENL in 2012 by Ministry of Health, Labour and Welfare. Use of thalidomide for patients with ENL has already been established by various studies in other countries, but limited experience in Japan has hindered application of this medication to domestic patients. This led us to devise a guideline on the usage of thalidomide to treat ENL in Japan. Based on TERMS, we suggest that administration of thalidomide for ENL should be started at 50-100 mg/day p.o. before going to bed and then the dose should be adjusted according to the patient’s symptoms, while not exceeding the maximum recommended dose of 300 mg/day. Introduction In Japan, the treatment of leprosy (Hansen’s disease) has been covered by the national health insurance scheme and performed at general hospitals since the Leprosy Prevention Law was abolished in 1996. Type 2 lepra reactions (also known as erythema nodosum leprosum: ENL), which often occur during the treatment of leprosy, are also managed at general hospitals. Under these circumstances, we developed this guideline for the proper use of thalidomide, which is currently thought to be useful for the treatment of ENL based on global data. The pathophysiology of ENL has not been fully elucidated. There is little clinical experience and no strong evidence supporting the use of thalidomide for ENL, as there has been no clinical trials testing thalidomide for ENL. This guideline presents expert opinion on the use of thalidomide based on best available data in order to support clinical decision of physicians. Physicians are encouraged to adapt their management based on their patient’s clinical presentation. Thalidomide Education and Risk Management System (TERMS) must be adhered when using thalidomide in Japan. Furthermore, physicians using thalidomide should always read the “Message from thalidomide victims” at the end of this guideline and keep in mind the lessons learned from the history of thalidomide embryopathy (phocomelia, etc.). 1) Leprosy and lepra reactions Leprosy is a chronic infection caused by the acid-fast bacterium Mycobacterium leprae. Acute inflammatory symptoms that can arise during the course of this disease are called lepra reactions. There are 2 types of lepra reactions, which ary type 1 reaction (reversal reaction) and type 2 reaction (ENL) based on the different pathophysiology. Type 1 reaction is known to be associated with delayed type hypersensitivity while type 2 reaction is antibody response to M. leprae. Both types of reactions may continue for long time and are closely related to peripheral neuropathy and its sequelae, impairment of vision, so it is necessary to make an early diagnosis and provide appropriate treatment. 2) Thalidomide Thalidomide has a molecular formula of C13H10N2O4 and its chemical name is 2-[(3RS)-2,6-dioxopiperidin3-yl] isoindoline-1,3-dione. It was synthesized in Switzerland in 1953, and was first marketed as a sedative/hypnotic in West Germany in 1957. Thalidomide was also released in Japan in 1958. It was subsequently found that taking thalidomide during early pregnancy could cause severe birth defects and Grünenthal GmbH (West Germany) initiated a product recall in November 1961. In Japan, sales were stopped and product recall was initiated in September 1962, but some of the products remained on the market after that time. It was reported that there were 309 thalidomide victims in Japan. An application for marketing thalidomide in the United States (US) was made in 1960, but Food and Drug Administration (FDA) examiner Frances Kelsey questioned its safety and the drug was not approved, at that time. It was fortuitously discovered that thalidomide could improve the symptoms of ENL when patients with leprosy took this drug. In 1998, thalidomide was approved in the US as a treatment for ENL (Table 1), followed by approvals in Australia and New Zealand. In 1998, thalidomide was also reported to be effective for multiple myeloma, and it was approved for

Guideline for clinical use of thalidomide for management of erythema nodosum leprosum in Japan