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Medications received by patients with juvenile dermatomyositis.

ABSTRACT: OBJECTIVE:Few controlled studies are available to guide treatment decisions in juvenile dermatomyositis (JDM). This study evaluated therapies received, changes of treatment over time, and factors associated with medication choices in JDM. METHODS:We performed a retrospective analysis of the number and type of therapies and duration of treatment for 320 patients with JDM enrolled in a North American registry. Kaplan-Meier and logistic regression analysis were used to assess the association of demographic and clinical features and autoantibodies with medication usage. RESULTS:High-dose oral prednisone was the primary therapy given to 99% of patients. 1997 was determined to be a threshold year for increasing usage of medications other than prednisone. The median time to half the initial oral prednisone dose was shorter in patients diagnosed after vs. before 1997 (10 vs. 19 months, P < 0.01). Patients received intravenous methylprednisolone (IVMP), methotrexate, intravenous immunoglobulin, antimalarial drugs, and combination therapy more frequently when diagnosed after 1997. IVMP was frequently received by patients with severe illness onset, anti-p155/140 (anti-TIF1) and anti-MJ (anti-NXP2) autoantibodies. Treatment with methotrexate was associated with older age at diagnosis and anti-MJ autoantibodies, while antimalarial therapy was associated with anti-p155/140 autoantibodies and mild onset severity. CONCLUSION:Oral prednisone has been the mainstay of therapy in JDM, and prednisone was reduced faster in patients diagnosed after 1997 when there was also an increase in other medications. Specific medications received by patients with JDM correlated with year and age at diagnosis, myositis autoantibodies, onset severity, and illness features.

SUBMITTER: Kishi T

PROVIDER: S-EPMC6162169 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature

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Medications received by patients with juvenile dermatomyositis.

Kishi Takayuki T Bayat Nastaran N Ward Michael M MM Huber Adam M AM Wu Lan L Mamyrova Gulnara G Targoff Ira N IN Warren-Hicks William J WJ Miller Frederick W FW Rider Lisa G LG

Seminars in arthritis and rheumatism 20180328 3

<h4>Objective</h4>Few controlled studies are available to guide treatment decisions in juvenile dermatomyositis (JDM). This study evaluated therapies received, changes of treatment over time, and factors associated with medication choices in JDM.<h4>Methods</h4>We performed a retrospective analysis of the number and type of therapies and duration of treatment for 320 patients with JDM enrolled in a North American registry. Kaplan-Meier and logistic regression analysis were used to assess the ass ...[more]

PMID: 29773230

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Project description:Background:Blood accessible biomarkers to assess disease activity and their response to therapies in Juvenile Dermatomyositis (JDM) are urgently needed. This pilot study aims to identify serum protein biomarkers associated with clinical disease activity in untreated JDM and their response to medical therapy. Methods:SomaScan® technology screened JDM patients for 1305 proteins at three points: 1) before start of treatment, 2) while on therapy, and 3) after treatment tapering when patients were clinically inactive. To define disease associated biomarkers, SomaScan® data from untreated JDM patients (n?=?8) were compared to SomaScan® data from an independent age-matched healthy control group (n?=?12). Longitudinal analysis defined treatment responsive proteins at three time points: untreated (7 samples), treated (7 samples), and clinically inactive (6 samples). To confirm the SomaScan® data, a subset of nine candidate proteins (CXCL11, IL-17B, IL-17D, IL-22, CXCL10, MCP-1, ANGPT2, MIF, IL-23) were tested by ELISA after adding 2 JDM (one untreated, one clinically inactive) serum samples to the same group of JDM girls (8 untreated, 7 treated; 7 clinically inactive) as well as with 17 age, gender, matched healthy controls. Results:Comparison of untreated JDM versus healthy controls identified 202 elevated and 49 decreased serum proteins in JDM patients with an adjusted p-value <?0.001. Only 82 out of 251 identified biomarker candidates responded to treatment while 12 out of these 82 proteins returned to their original untreated disease levels upon therapy tapering. The ELISA testing of the untreated samples for nine candidate proteins confirmed previously known biomarkers (CXCL10 or IP-10, CXCL11 or I-TAC and MCP-1) and identified novel biomarkers including IL-22, Angiopoetin-2, and IL-17B in a cross-sectional analysis comparing 8 untreated JDM and 17 age/gender matched controls. The subsequent longitudinal data by ELISA were not concordant for some biomarkers (IL-22 and IL-17B), but the other biomarkers either normalized or rebounded concordantly. Conclusions:Blood accessible protein biomarkers reflecting JDM pathophysiology were identified; some of them rebounded after therapy was tapered. Further studies bridging these biomarkers to specific clinical features of JDM are required to confirm the clinical utility of these serum protein biomarkers.

Project description:BackgroundPrednisone (PDN) in juvenile dermatomyositis (JDM), alone or in association with other immunosuppressive drugs, namely methotrexate (MTX) and cyclosporine (CSA), represents the first-line treatment option for new onset JDM patients. No clear evidence based guidelines are actually available to standardize the tapering and discontinuation of glucocorticoids (GC) in JDM. Aim of our study was to provide an evidence-based proposal for GC tapering/discontinuation in new onset juvenile dermatomyositis (JDM), and to identify predictors of clinical remission and GC discontinuation.MethodsNew onset JDM children were randomized to receive either PDN alone or in combination with methotrexate (MTX) or cyclosporine (CSA). In order to derive steroid tapering indications, PRINTO/ACR/EULAR JDM core set measures (CSM) and their median absolute and relative percent changes over time were compared in 3 groups. Group 1 included those in clinical remission who discontinued PDN, with no major therapeutic changes (MTC) (reference group) and was compared with those who did not achieve clinical remission, without or with MTC (Group 2 and 3, respectively). A logistic regression model identified predictors of clinical remission with PDN discontinuation.ResultsBased on the median change in the CSM of 30/139 children in Group 1, after 3 pulses of methyl-prednisolone, GC could be tapered from 2 to 1 mg/kg/day in the first two months from onset if any of the CSM decreased by 50-94%, and from 1 to 0.2 mg/kg/day in the following 4 months if any CSM further decreased by 8-68%, followed by discontinuation in the ensuing 18 months. The achievement of PRINTO JDM 50-70-90 response after 2 months of treatment (ORs range 4.5-6.9), an age at onset > 9 years (OR 4.6) and the combination therapy PDN + MTX (OR 3.6) increase the probability of achieving clinical remission (p < 0.05).ConclusionsThis is the first evidence-based proposal for glucocorticoid tapering/discontinuation based on the change in JDM CSM of disease activity.Trial registrationTrial full title: Five-Year Single-Blind, Phase III Effectiveness Randomized Actively Controlled Clinical Trial in New Onset Juvenile Dermatomyositis: Prednisone versus Prednisone plus Cyclosporine A versus Prednisone plus Methotrexate. EUDRACT registration number: 2005-003956-37 .Clinical trialgov is NCT00323960 . Registered on 17 August 2005.

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Medications received by patients with juvenile dermatomyositis.

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Medications received by patients with juvenile dermatomyositis.

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