Juvenile localized scleroderma: can we modify treatment strategies based on predictors of relapse?

2019 ◽  
Vol 180 (5) ◽  
pp. 990-991
Author(s):  
C. E. Pain

Rheumatology ◽  
2005 ◽  
Vol 45 (5) ◽  
pp. 614-620 ◽  
Author(s):  
F. Zulian ◽  
B. H. Athreya ◽  
R. Laxer ◽  
A. M. Nelson ◽  
S. K. Feitosa de Oliveira ◽  
...  


2017 ◽  
Vol 24 (3) ◽  
pp. 145-152
Author(s):  
Christine Arango ◽  
Clara Malagón ◽  
María del Pilar Gómez ◽  
Catalina Mosquera ◽  
Ricardo Yépez ◽  
...  


2010 ◽  
Vol 55 (3) ◽  
pp. 308 ◽  
Author(s):  
PrabhakarM Sangolli ◽  
NC Hiremath ◽  
NT Madan Mohan ◽  
C Srinivas ◽  
K Srinivas ◽  
...  




2019 ◽  
Vol 47 (8) ◽  
pp. 1242-1252 ◽  
Author(s):  
Suzanne C. Li ◽  
Kathryn S. Torok ◽  
C. Egla Rabinovich ◽  
Fatma Dedeoglu ◽  
Mara L. Becker ◽  
...  

Objective.To perform a comparative effectiveness feasibility study in juvenile localized scleroderma (LS), using standardized treatment regimens (consensus treatment plans; CTP).Methods.A prospective, multicenter 1-year pilot observational cohort study was performed by Childhood Arthritis and Rheumatology Research Alliance (CARRA) LS workgroup members. Patients with active, moderate to severe juvenile LS were treated with one of 3 CTP: methotrexate alone, or in combination with intravenous (30 mg/kg/dose for 3 mos) or oral corticosteroids (2 mg/kg/day tapered by 48 weeks).Results.Fifty patients, with demographics typical for juvenile LS, were enrolled, and 44 (88%) completed the study. Most had extracutaneous involvement. Patients improved in all 3 CTP, with > 75% having a major or moderate level of improvement compared to baseline. Damage accrued in some patients. Major deviations from prescribed regimen resulted from medication intolerance (n = 6; 14%) or treatment failure (n = 11; 25%); failures occurred in all 3 CTP. Significant responses to treatment were demonstrated by LS skin scoring measures and overall physician assessments, with differences in response level identified in some patient subsets. Response differences were associated with baseline disease activity level, LS subtype, skin disease extent, and extracutaneous involvement.Conclusion.This study demonstrates the feasibility of conducting juvenile LS comparative effectiveness studies. The CTP were found to be safe, effective, and tolerable. Our assessments performed well. Because damage is common and may progress despite effective control of activity, we recommend initial treatment efficacy be evaluated primarily by activity measures. Potential confounders for response were identified that warrant further study.



2018 ◽  
Vol 12 (1) ◽  
pp. 21-23 ◽  
Author(s):  
Mackenzie L. Franklin ◽  
Shelley Day


2017 ◽  
Vol 69 (7) ◽  
pp. 1082-1087 ◽  
Author(s):  
Kaveh Ardalan ◽  
Christina K. Zigler ◽  
Kathryn S. Torok


2009 ◽  
Vol 37 (1) ◽  
pp. 175-181 ◽  
Author(s):  
SUZANNE C. LI ◽  
BRIAN M. FELDMAN ◽  
GLORIA C. HIGGINS ◽  
KATHLEEN A. HAINES ◽  
MARILYNN G. PUNARO ◽  
...  

Objective.We surveyed pediatric rheumatologists (PR) in North America to learn how they treat pediatric localized scleroderma (LS), a disease associated with significant morbidity for the growing child.Methods.A Web-based survey was sent to the 195 PR members of the pediatric rheumatology research alliance CARRA (Childhood Arthritis and Rheumatology Research Alliance). Members were asked which medications they use to treat LS and which factors modify their treatment strategies. Clinical vignettes were provided to learn the specific treatment regimens used.Results.A total of 158 PR from over 70 clinical centers in the United States and Canada participated in the survey, representing 81% of the CARRA membership. These PR saw over 650 patients with LS in the prior year. Nearly all respondents treated LS with methotrexate (MTX) and corticosteroids; most of them intensify treatment for lesions located on the face or near a joint, and about half intensify treatment for recent disease onset (< 6 months). Most PR reserve topical medications for limited treatment situations. Clinical vignettes showed that PR use a broad range of treatment doses and durations for MTX and corticosteroids.Conclusion.Most PR in North America treat localized scleroderma with a combination of MTX and corticosteroids. However, there is no consensus on specific treatment regimens. There is a need for controlled treatment trials to better determine optimal therapy for this potentially disabling disease.



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