000 03624nam a22004937a 4500
008 141002s20142014 xxu||||| |||| 00| 0 eng d
022 _a0315-162X
040 _aOvid MEDLINE(R)
099 _a24293577
245 _aClinical features of patients with morphea and the pansclerotic subtype: a cross-sectional study from the morphea in adults and children cohort.
251 _aJournal of Rheumatology. 41(1):106-12, 2014 Jan.
252 _aJ Rheumatol. 41(1):106-12, 2014 Jan.
253 _aThe Journal of rheumatology
266 _d2014-10-02
501 _aAvailable online from MWHC library: 2001 - present
520 _aCONCLUSION: Our results suggest demographic and clinical features are sufficient to define the pansclerotic subtype as they represent a distinct clinical phenotype with a more rapidly progressive and severe course commonly accompanied by disability. Presence of features of the pansclerotic phenotype should alert practitioners to the possibility of significant morbidity and the need for early aggressive treatment.
520 _aMETHODS: Patients who met predefined criteria for generalized and pansclerotic morphea were identified using a modified Laxer and Zulian classification system. Baseline demographic and clinical features of the patients were compiled and then analyzed for traits characteristic of pansclerotic morphea versus those of generalized morphea. One hundred and thirteen patients met the criteria for inclusion: pansclerotic (n = 13) and generalized morphea type (n = 100).
520 _aOBJECTIVE: Pansclerotic morphea is a poorly described form of morphea with little information on prevalence, demographics, and clinical features. Classification criteria for this subtype varies and the distinction from other forms of morphea, such as extensive generalized morphea and pansclerotic morphea, is not always clear. The purpose of our study was to clarify classification criteria for pansclerotic morphea by identifying its prevalence in the morphea in adults and children (MAC) cohort and describing its demographic and clinical features as compared with generalized morphea.
520 _aRESULTS: Patients with pansclerotic morphea were more frequently male (46.2% vs 6%; p < 0.0001); had a shorter time to diagnosis (mean difference of 10.4 mos; 95% CI: 0.8-19.9 mos; p = 0.0332); higher rates of functional impairment (61.5% vs 16%; p = 0.0046); higher rates of deep involvement (61.5% vs 17%; p = 0.004); and higher average Rodnan Skin Score (mean difference of 10.8 points; 95% CI: 5-16.6; p = 0.0017), Localized Scleroderma Skin Damage Index (mean difference 28.3; 95% CI: 9-47.6; p = 0.009), and Physician Global Assessment of Disease Damage scores (mean difference 25.1; 95% CI: 0.3-50; p = 0.048).
546 _aEnglish
650 _a*Scleroderma, Localized/di [Diagnosis]
650 _aAdult
650 _aAged
650 _aChild
650 _aCross-Sectional Studies
650 _aDisabled Persons
650 _aFemale
650 _aHumans
650 _aMale
650 _aMiddle Aged
650 _aRegistries
650 _aSeverity of Illness Index
650 _aSex Factors
650 _aTime Factors
650 _aYoung Adult
651 _aMedStar Washington Hospital Center
656 _aDermatology
657 _aJournal Article
657 _aResearch Support, N.I.H., Extramural
700 _aMarinkovich, Nicholas
790 _aJacobe HT, Kim A, Marinkovich N, Vasquez R
856 _uhttp://dx.doi.org/10.3899/jrheum.130029
_zhttp://dx.doi.org/10.3899/jrheum.130029
942 _cART
_dJournal article
999 _c12263
_d12263