Japan's health ministry wants to add six conditions to its list of designated intractable diseases, the list that decides whose medical costs qualify for specific medical expense payments, and to rename four existing entries. The Health and Living Hygiene Bureau's Intractable Disease Control Division opened public comment on the draft notification on September 28, 2026, and takes submissions until October 29.
The list already holds 348 diseases. The draft numbers the new entries 349 to 354. The ministry says the changes follow the 67th and 68th meetings of its designated intractable disease review committee, which judged the renames and additions appropriate.
The six additions
The new entries are retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S), Schnitzler syndrome, congenital platelet function disorders (Glanzmann thrombasthenia and Bernard-Soulier syndrome), congenital bile acid metabolism disorders (excluding cerebrotendinous xanthomatosis), idiopathic peripheral corneal ulcer and VEXAS syndrome.
The annexes give each disease its own diagnostic criteria and severity cut-off, and the patient counts are small.
| Disease | Patient figure in annex | Coverage threshold |
|---|---|---|
| RVCL-S | Fewer than 10 | Moderate or above |
| Schnitzler syndrome | About 50 (research group) | Severe cases |
| Congenital platelet function disorders | 1986 national survey: 222 Glanzmann thrombasthenia, 43 Bernard-Soulier syndrome | Moderate or above |
| Congenital bile acid metabolism disorders (excluding cerebrotendinous xanthomatosis) | 12 (known to research group) | Severity 2 or above |
| Idiopathic peripheral corneal ulcer | About 500 to 1,000 (2010 survey) | Grade III or above |
| VEXAS syndrome | Fewer than 1,000 (July 2026 research survey) | Meets any one of three research-group criteria |
Every annex adds that a patient who falls below its severity cut-off but needs continued high-cost care is still eligible for the subsidy.
The renames
Sjögren's syndrome becomes Sjögren's disease. Three vascular and lymphatic malformation entries affecting the neck and face, mouth and throat, or limbs change only a variant character in the word for neck. The annex for Sjögren's disease keeps its 1999 research-group criteria and targets patients scoring 5 or more on the ESSDAI activity index.
Timing
The ministry plans to issue the notification in late March 2027, with an application date of April 1, 2028. The circular to prefectures and designated cities, which carries the new diagnostic criteria and severity classifications, is also planned for March 2027 and would apply to payment approvals made on or after April 1, 2028.
Comments must be in Japanese, with reasons, through the e-Gov form, by email or by post to the division. The ministry says it will not reply to individual submissions and may publish comments other than contact details.