Background/Purpose: Hydroxychloroquine is an antimalarial agent commonly used in the treatment of rheumatologic diseases. Data on the use of HCQ in JDM is limited, primarily based on anecdotal experience and two small, retrospective reviews. As a result, there is no current consensus on the utility of HCQ in the management of JDM. Our objective was to investigate the use of HCQ in JDM using a large, national, multi-center registry.

Methods: Subjects meeting Bohan and Peter criteria for definite or probable JDM were enrolled into the CARRA registry between 2010 and 2013. Cross sectional analysis of data regarding demographics, disease characteristics, measures of disease activity, diagnostic assessments, medications and long term complications were examined. Bivariate analysis was performed evaluating the use of HCQ with clinical variables of interest. Variables with biologic relevance or statistically significant associations (p<0.25) were selected for inclusion into a multivariate logistic regression model examining independent predictors of HCQ use.

Results: Baseline information was available for 604 patients. Information on HCQ treatment was available for 565 patients (93.5%). 295 (52.2%) patients had current or prior treatment with HCQ and 270 (47.8%) patients had never been treated with HCQ. In bivariate analysis, an association was found between the use of HCQ and age at first rheumatology visit (p=0.02), disease duration (p<0.001), abnormal CHAQ (p=0.001), symmetric proximal muscle weakness (p=0.002), large joint arthritis (p=0.002), malar erythema (p=0.04) and calcinosis (p=0.012). In multivariate analysis, patients with symmetrical proximal muscle weakness or an abnormal CHAQ score were less likely to be on HCQ therapy; whereas patients with any rash (malar erythema, Gottron’s sign, heliotrope rash, V-sign or shawl sign), calcinosis or arthritis had a higher likelihood to be on current or prior HCQ therapy (Table 1).

Table 1. Multivariable model using logistic regression predicting use of HCQ (n=496)

	Odds ratio [95% Confidence interval]	p- value
Symmetrical muscle weakness	0.46 [0.28-0.74]	0.002
Abnormal CHAQ	0.66 [0.44-0.99]	0.044
Calcinosis	1.93 [1.08-3.45]	0.027
Rash	1.65 [1.11-2.46]	0.014
Arthritis	1.56 [1.05-2.31]	0.027

Conclusion: The CARRA registry represents one of the largest ongoing multi-center JDM registries. This data aids our understanding of which clinical characteristics may predict HCQ use in JDM. Prior evidence in other rheumatologic conditions suggests that HCQ improves arthritis and skin manifestations. As expected, patients with these characteristics were more likely to be on HCQ. Patients with muscle weakness and abnormal CHAQ were less likely to be on HCQ. We speculate these patients had a more severe disease course and likely treated with aggressive immunosuppression. Due to the design of this study as a cross-sectional analysis, we are unable to determine the causal association of these results. Further longitudinal data is needed to examine the benefits of HCQ in these patients.

Funded by the NIAMS, Friends of CARRA, CARRA Inc., and the Arthritis Foundation.

---

« Back to 2014 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/cross-sectional-evaluation-of-hydroxychloroquine-hcq-therapy-in-children-with-juvenile-dermatomyositis-jdm-enrolled-in-the-childhood-arthritis-and-rheumatology-research-alliance-carra-regis/