Response to colchicine has been variable. 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. Stem cell transplants have been given to saves lives.
Haploinsufficiency of A20
Chromosome 2q deletion
Y chromosome microdeletion
Prenatal cocaine exposure
Mevalonate kinase deficiency
17β-Hydroxysteroid dehydrogenase III deficiency
WNT4 deficiency
Guanidinoacetate methyltransferase deficiency