The age on onset is also variable ranging from the first week of life to 29 years.

[citation needed] It appears that two copies of this gene are required to avoid inflammatory features developing - hence the name haploinsufficiency.

[citation needed] The main differential diagnosis are Behçet's disease and systemic lupus erythematosus.

Cytokine inhibitors - including the anti-IL6 receptor biologic tocilizumab - appear to be the most effective.Given the rarity of this condition, optimal management has not yet been definitely identified.

[citation needed] Stem cell transplants have been given to saves lives.