Prospective, longitudinal studies examining the features of linear morphea are limited.
To utilize the Morphea in Adults and Children cohort to determine clinical characteristics, impact on life quality, and disease course of linear morphea in a prospective, longitudinal manner.
Characteristics of linear morphea versus other subtypes were compared in a cross-sectional manner. Next, linear morphea participants were examined in depth over a 3-year period.
Linear morphea was the most common morphea subtype (50.1%, 291/581) in the cohort. Deep involvement was more common in linear (64.3%, 187/291) than other morphea subtypes. Linear morphea participants with deep involvement were more likely to have a limitation in range of motion (28.6%, 55/192) than those without (11.1%, 11/99, P < .001). Adult-onset disease occurred in 32.6% (95/291) of those with linear morphea. Frequency of deep involvement was similar between pediatric (66.8%, 131/196) and adult-onset linear morphea (58.9%, 56/95, P = .19). Quality of life and disease activity scores improved over time, while damage stabilized with treatment.
Results of the study are associative, and the University of Texas Southwestern Medical Center is a tertiary referral center.
A substantial number of linear morphea patients have adult-onset disease. In all age groups, linear morphea with deep involvement was associated with functional limitations.Le texte complet de cet article est disponible en PDF.
Key words : en coup de sabre, linear morphea, localized scleroderma, MAC cohort, Morphea in Adults and Children cohort, Parry-Romberg syndrome, quality of life
Abbreviations used : CDLQI, DLQI, IQR, MAC, LoSCAT, mLoSSI, LoSDI, PGA-A, PGA-D
| Dr Kunzler and Ms Florez-Pollack are co-first authors.
| Funding sources: Supported by the National Center for Advancing Translational Sciences of the National Institutes of Health under award number TL1TR001104.
| Conflicts of interest: None disclosed.
| Disclaimer: The content is solely the responsibility of the authors and does not necessarily represent the official views of the University of Texas Southwestern Medical Center at Dallas or its affiliated academic and health care centers or the National Institutes of Health.
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