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+The patient, a 4-year-old boy, was diagnosed as having Blau syndrome based on the manifestation of typical clinical features (ankle and wrist arthritis/tenosynovitis, diffuse eczematous rash, and uveitis), histologic evidence of noncaseating granulomas, and a heterozygous NOD2 mutation (p.R334W).
+Ocular involvement was initially controlled by topical and oral corticosteroids, but over the years visual impairment progressed.
+Other manifestations of Blau syndrome (arthritis and rash) subsided over time.
+Bilateral panuveitis progressed after age 5 years, and was initially treated with methotrexate.
+However, ocular inflammation persisted despite the addition of local steroid injections and repeated intravenous (IV) bolus methylprednisolone treatment; therefore, when the patient was age 10 years, infliximab (initially at 5 mg/kg increased to 10 mg/kg IV every 4 to 6 weeks) was initiated.
+Although there was an initial improvement, 1 year after this treatment was started uveitis worsened, and at age 12 years infliximab was discontinued.
+Adalimumab (24 mg/m2 every 2 weeks) was then initiated and the dosage of methotrexate (15 mg/m2/weekly) was increased.
+However, ocular disease remained active.
+Mycophenolate mofetil (750 mg/m2) and then abatacept (10 mg/kg/month IV) were sequentially administered, without significant improvement.
+At age 16 years the patient still had granulomatous retinal lesions and anterior chamber inflammation, and macular edema developed, which led to retinal detachment.
+In addition to the other steroid therapy, corticosteroid pulse therapy was necessary to control disease flares, with an average of 3 boluses/month for 6 consecutive months.
+Because of the supposed autoinflammatory nature of Blau syndrome, we initiated a trial of IL-1 antibody administration (2 mg/kg/month of canakinumab).
+During the 6 months that followed, no ocular flare occurred and no steroid pulse therapy was necessary.
+Concomitant treatment with oral methotrexate and low-dose prednisone (0.2 mg/kg/day) remained unchanged.
+Figure 1 shows fluorangiograms before treatment and after the first 6 injections.
+The drug was well tolerated with no side effects, and findings on laboratory tests (performed monthly) were normal.