Pediatric Rheumatology Online Journal → August 2003 → Newer Treatments → Anti-Tumor Necrosis Factor Therapy → Abstract #160

EXPANDED REVIEW OF INFLIXIMAB THERAPY FOR STEROID-DEPENDENT EARLY ONSET SARCOID ARTHRITIS AND BLAU SYNDROME: 5 PEDIATRIC CASES

A. C. Brescia,¹ G. McIlvain-Simpson,¹ C. D. Rose.¹

¹Pediatric Rheumatology, A.I. duPont Hospital for Children, Wilmington, DE

Early onset sarcoid arthritis (SA) requires for diagnosis polyarticular "boggy" synovitis with non-caseating granuloma, salmon-colored rash with dermal granuloma, uveitis and onset before age five years. Blau Syndrome is an autosomal dominant familial form of the disease. The arthritis and uveitis are often refractory to therapy, requiring prolonged use of steroids. We expand our recent description of the treatment of one child with SA and two children with BS with infliximab infusions (5 mg/kg) to include two further patients with BS.
Case 1, a 10 y.o. male with symptoms of BS since age 2 yrs, had arthritis refractory to NSAIDs and methotrexate, requiring steroids for 7 yrs. Infliximab infusions every two months for 6 doses without adverse reactions allowed every other day steroids and improved joint symptoms. Case 2 has required methotrexate and 8 yrs of prednisone. After 5 doses of infliximab steroid dose has been decreased by more than 50%. Case 3 has improved joint swelling and has tapered to every other day steroids after 5 infusions. Case 4 required prosthetic left eye from complications of refractory pan-uveitis. He has been treated with methotrexate, imuran, IVIg and 11 years of prednisone. After six infusions of infliximab, there is remarkable improvement in joint swelling, energy level, and uveitis. Case 5 is a 17 y.o. male with symptoms of SA since 6 months of age. Arthritis has been refractory to NSAIDs and colchicine, requiring 14 years of systemic steroids. Partial response was seen on etanercept. Systemic steroids have been tapered to low doses and joint swelling is at a minimum after 11 infusions.
Infliximab is well tolerated and affords dramatic improvement in pediatric patients with refractory steroid-dependent arthritis and uveitis associated with Blau Syndrome and Sarcoid Arthritis. Although mechanisms of these diseases remain elusive, TNFa is a powerful mediator of granuloma formation. This may explain the observed efficacy of TNF blockade with infliximab in these diseases.