Pediatric Rheumatology Online Journal → July 2003 → Vasculitides → Miscellaneous Vasculitis Entities → Abstract #141
                                                                August 2003 → Newer Treatments → Anti-Tumor Necrosis Factor Therapy → Abstract #141

INFLIXIMAB IN REFRACTORY BEHCET'S DISEASE IN CHILDREN

Y. Kimura.¹

¹Pediatric Rheumatology, Children's Hospital, Hackensack University Medical Center, Hackensack, NJ

Recalcitrant oral and genital ulcerations in Behcet's disease causes considerable disability and morbidity. TNF appears to be an important cytokine in this disease, and there have been a few case reports of adults with Behcet's successfully treated with anti-TNF therapy. We describe two pediatric patients with severe refractory mucocutaneous Behcet's who were successfully treated with infliximab.
Case 1: An 11 yo male presented with severe mouth ulcers, arthritis and nodular skin lesions and was diagnosed with Behcet's. He initially responded to high dose steroids but had severe recurrences with steroid tapering despite colchicine, pentoxifylline, and methotrexate. Eventually, he became refractory to steroids (including pulse methylprednisolone). He was treated with infliximab 5mg/kg. The ulcers healed within several days after the dose, and prednisone was able to be discontinued by the 4th dose, although lengthening the treatment intervals to 8 wks has resulted in mild recurrences which has required intermittent low-dose prednisone.
Case 2: A 13 yo female was diagnosed with Behcet's when she presented with oral and genital ulcers and femoral/iliac deep vein thrombosis. She was treated for 3 years with high dose prednisone, colchicine and azathioprine, but continued with debilitating ulcers and inability to taper steroids. She also developed pseudotumor cerebri which required lumboperitoneal shunting. She presented to our institution for the first time at age 16 with a large chronic ulcer overlying the area where 5 months previously the shunt had been removed, a large vulvar abscess and multiple large oral ulcers. She was given methotrexate and 10mg/kg of infliximab in addition to her prednisone. This resulted in complete resolution of the oral and vaginal ulcers/abscess. The ulcer on her back healed by the 3rd dose. She continues on monthly infliximab and methotrexate and is tapering off prednisone.
Conclusion: Anti-TNF therapy was effective and well-tolerated in these two pediatric patients with refractory Behcet's disease and may be a useful adjunctive therapy in other patients.