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Treatment of Pediatric Takayasu Arteritis With Infliximab and Cyclophosphamide: Experience From an American-Brazilian Cohort Study

Stern, Sara MD*; Clemente, Gleice MD; Reiff, Andreas MD; Ramos, Margarida Paula Romão MD; Marzan, Katherine Anne MD; Terreri, Maria Teresa MD

JCR: Journal of Clinical Rheumatology: June 2014 - Volume 20 - Issue 4 - p 183–188
doi: 10.1097/RHU.0000000000000106
Original Articles

Background Pediatric Takayasu arteritis (pTA) is difficult to treat and can lead to significant morbidity and mortality.

Objectives The objective of this study was to describe clinical characteristics for pTA and determine the safety and efficacy of cyclophosphamide (CYC) and infliximab (IFX) in pTA.

Methods This was a retrospective analysis of 23 pTA patients seen at Children’s Hospital Los Angeles and Universidade Federal de São Paulo–Brazil from 1990 to 2011. All patients fulfilled the 1990 American College of Rheumatology criteria for Takayasu arteritis. Disease activity was assessed using a modified National Institutes of Health score.

Results Twenty-three patients (14 female and 9 male patients), mean age of 15.7 ± 6.0 years, were included. Cyclophosphamide was used before IFX treatment in 17 of 23 and IFX before CYC in 2 of 23 patients. The average time from disease onset to treatment initiation was 2.6 ± 2.4 years for CYC and 4.1 ± 2.4 years for IFX. Nine (60%) of 15 patients failed CYC, and of these 6 were changed to IFX with subsequent clinical stabilization in 5 (83%) of 6. Two patients initially treated with IFX were switched to CYC because of lack of appropriate response: 1 patient later worsened, and the other was lost to follow-up. Five opportunistic infections requiring hospitalization occurred in the CYC group, whereas none were observed in the IFX group. Patients in the IFX group were more likely to decrease or stop their corticosteroids when compared with the CYC patients.

Conclusions Cyclophosphamide is often used as initial treatment but has many adverse effects. In this retrospective case series, IFX was equivalent to CYC with fewer adverse effects, making IFX an alternative therapeutic option for pTA.

From the *Division of Immunology, Rheumatology, Allergy, University of Utah, Salt Lake City, UT; †Pediatric Rheumatology Unit, Pediatric Department, Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil; and ‡Division of Rheumatology, University of Southern California, Children’s Hospital Los Angeles, Los Angeles, CA.

This study has no sources of support.

Correspondence: Sara Stern, MD, University of Utah, Department of Pediatrics, PO Box 581289, Salt Lake City, UT 84158. E-mail:

© 2014 by Lippincott Williams & Wilkins, Inc.