Protocols for the initial treatment of moderately severe juvenile dermatomyositis: results of a Children's Arthritis and Rheumatology Research Alliance Consensus Conference.

OBJECTIVE: To use juvenile dermatomyositis (DM) survey data and expert opinion to develop a small number of consensus treatment protocols, which reflect current initial treatment of moderately severe juvenile DM. METHODS: A consensus meeting was held in Toronto, Ontario, Canada on December 1-2, 2007. Nominal group technique was used to achieve consensus on treatment protocols, which represented typical management of moderately severe juvenile DM. Consensus was also reached as to which patients these protocols would be applicable (inclusion and exclusion criteria), which initial investigations should be done prior to initiating one of these protocols, which data should be collected to evaluate these protocols, and the concomitant interventions required or recommended. RESULTS: Three protocols that described the first 2 months of treatment were developed. All protocols included corticosteroids and methotrexate. One protocol also included intravenous gamma globulin. Consensus was achieved for all issues that were addressed by conference participants, although there were some areas of controversy. CONCLUSION: Despite considerable variation in clinical practice, it is possible to achieve consensus on the initial treatment of juvenile DM. Once these protocols are extended beyond 2 months, these protocols will be available for clinical use. By using methods that account for differences between patients (confounding by indication), the comparative effectiveness of the protocols will be evaluated. In the future, the goal will be to identify the optimal treatment of moderately severe juvenile DM.

Incomplete Kawasaki disease in patients younger than 1 year of age: a possible inherent risk factor.

Kawasaki disease (KD) patients younger than 1 year of age are at especially high risk of developing coronary artery abnormalities (CAA). To define the clinical characteristics of this group, as well as the risk factors predisposing them to CAA, we reviewed the medical records of 136 KD patients younger than 1 year of age who were treated at the Korea University Medical Center from January 2001 to July 2006. Of these patients, 16 developed CAA (11.8%). The CAA(+) group had a longer duration of total fever than the CAA(-) group (9.1+/-3.7 days vs. 6.3+/-2.0 days, p=0.011), but did not differ in the duration of pre- and post-intravenous gamma-globulin (IVGG) fever. The CAA(+) group had fewer diagnostic symptoms than the CAA(-) group (2.7+/-1.1 vs. 4.3+/-1.2, p<0.001). Of the hematological findings, the CAA(+) group only differed from the CAA(-) group in having significantly higher total white blood cell (19.2+/-6.0 vs. 14.7+/-4.7 K/mm(3), p=0.007) and platelet (462.9+/-101.0 vs. 383.6+/-121.1 K/mm(3), p=0.014) levels. Multivariable logistic regression analysis showed that the only factors which were significantly associated with the development of CAA were the total number of symptoms (OR=0.493, 95% CI=0.293-0.829, p=0.007) and the duration of total fever (OR=1.405, 95% CI=1.092-1.808, p=0.008). Conclusively, incomplete clinical manifestations and a longer duration of total fever are significantly associated with the development of CAA in KD patients younger than 1 year of age. Therefore, these patients should be monitored for incomplete KD, especially if unexplained fever continues, and treatment to shorten the duration of total fever should be initiated.