The Localized Scleroderma Skin Severity Index and Physician Global Assessment of Disease Activity: A Work in Progress Toward Development of Localized Scleroderma Outcome Measures

Arkachaisri, Thaschawee; Vilaiyuk, Soamarat; Li, Suzanne; O’Neil, Kathleen M.; Pope, Elena; Higgins, Gloria C.; Punaro, Marilynn; Rabinovich, Egla; Rosenkranz, Margalit; Kietz, Daniel; Rosen, Paul Peter; Spalding, Steven J.; Hennon, Teresa; Torok, Kathryn S.; Cassidy, Elaine; Medsger, Thomas A.

doi:10.3899/jrheum.081284

Cited by 162 publications

(202 citation statements)

References 43 publications

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“…Para a avaliação da atividade da doença, foram considerados parâmetros clínicos e laboratoriais usados na prática clínica diária [21][22][23][24] .…”

Section: Métodosunclassified

Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases

Bugni¹,

Ozaki²,

Okamoto³

et al. 2012

J Pediatr (Rio J)

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Objective: There are several factors that contribute to poor adherence to treatment in children and adolescents with chronic rheumatic diseases, worsening their quality of life and prognosis. Our aim was to assess the rates of adherence to treatment and to identify the socioeconomic and clinical factors associated. Methods:The sample included 99 patients with juvenile idiopathic arthritis, systemic erythematosus lupus, dermatomyositis or juvenile scleroderma. All patients were followed at the outpatient pediatric rheumatology for a minimum period of 6 months. To assess adherence, a questionnaire was administered to the providers, which included three blocks: 1) demographic, clinical and laboratory data; 2) medication adherence; and 3) attending follow-up appointments, examinations and use of orthoses. A value lower than or equal to 80% of the prescribed was considered poor adherence.Results: A total of 53% of patients showed good overall adherence, observed when the caregiver lived in a stable union marital status (p = 0.006); 20 patients (20.2%) presented poor medication adherence, related to the use of three or more medications daily (p = 0.047). The causes of poor adherence were forgetfulness, refusal, incorrect dose or lack of medication, personal problems, and financial difficulties. Conclusions:We observed good overall treatment adherence in patients whose providers lived in stable union and poor adherence to medication in patients who used more than three types of medication daily. There was no association between the adherence rates and sex, age, time since diagnosis and disease activity. J Pediatr (Rio J). 2012;88(6):483-8:Patient adherence, therapy, chronic disease, pediatrics, rheumatology. ResumoObjetivo: São vários os fatores que contribuem para a má adesão ao tratamento de crianças e adolescentes com doenças reumáticas crônicas, gerando piora da qualidade de vida e do prognóstico. Nosso objetivo foi avaliar as taxas de adesão ao tratamento e identificar os fatores socioeconômicos e clínicos associados. Métodos:Foram incluídos 99 pacientes com artrite idiopática juvenil, lúpus eritematoso sistêmico, dermatomiosite ou esclerodermia juvenil. Todos os pacientes eram acompanhados no ambulatório de reumatologia pediátrica por um período mínimo de 6 meses. Para avaliação da adesão, foi aplicado aos cuidadores um questionário composto por três blocos: 1) dados demográficos, clínicos e laboratoriais; 2) adesão ao tratamento medicamentoso; e 3) comparecimento às consultas, realização de exames e utilização de órteses. Foi considerada má adesão, quando realizado valor menor ou igual a 80% do prescrito.Resultados: Um total de 53% dos pacientes apresentou boa adesão ao tratamento global, observada quando o cuidador possuía união estável (p = 0,006); 20 pacientes (20,2%) apresentaram má adesão ao tratamento medicamentoso, relacionada à utilização de mais que três medicamentos diários (p = 0,047). As causas de má adesão ao tratamento foram esquecimento, recusa, dose incorreta ou falta de medicamento, prob...

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“…Para a avaliação da atividade da doença, foram considerados parâmetros clínicos e laboratoriais usados na prática clínica diária [21][22][23][24] .…”

Section: Métodosunclassified

Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases

Bugni¹,

Ozaki²,

Okamoto³

et al. 2012

J Pediatr (Rio J)

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“…Subiektywna ocena lekarska aktywności choroby (PGA-A) powinna bazować na cechach klinicznych, które zostały zakwalifikowane do trzech grup, w zależności od zgodności ocen (tab. 7) [47]. Uszkodzenie tkanek w przebiegu LoSc określa się na podstawie oceny atrofii skóry (0 -brak, 1 -niewielka atrofia lub skóra błyszcząca, 2 -umiarkowana atrofia lub prześwitujące naczynia, 3 -znaczna atrofia lub zagłębienie) i tkanki podskórnej (0 -brak, 1 -płytkie wgłębienie, utrata ≤ 1/3 tkanki tłuszczowej, 2 -wklęśnięcie, utrata 1/3-2/3 tkanki tłuszczowej, 3 -znaczna atrofia, utrata ≥ 2/3 tkanki tłuszczowej) oraz hipo-lub hiperpigmetacji (0 -brak, 1 -niewielka, 2 -umiarkowana, 3 -znaczna).…”

Section: Metody Oceny Aktywności Lub Nasilenia Choroby I Uszkodzenia unclassified

“…For a patient to be considered to have active disease, a single level 1 parameter or two level 2 parameters had to be identified [46]. Subjective physician assessment of disease activity (PGA-A) should be based on clinical variables classified into three groups depending on the consensus agreement (Table 7) [47]. Tissue damage secondary to LoSc is assessed on the basis of dermal atrophy (0 -none, 1 -mild atrophy/ shiny skin, 2 -moderate atrophy/visible vessels, 3 -marked atrophy/'cliffdrop') and subcutaneous atrophy (0 -none, 1 -flat, ≤ 1/3 fat loss, 2 -concave, 1/3-2/3 fat loss, 3 -marked atrophy, ≥ 2/3 fat loss), and hypo-/hyperpigmentation (0 -none, 1 -mild, 2 -moderate, 3 -marked).…”

Section: Metody Oceny Aktywności Lub Nasilenia Choroby I Uszkodzenia mentioning

confidence: 99%

Localized scleroderma – classification and tools used for the evaluation of tissue damage and disease activity/severity

Wolska-Gawron¹,

Krasowska²

2017

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Localized scleroderma is a rare autoimmune disorder affecting the dermis, subcutaneous tissue and deeper structures. The course of localized scleroderma includes three stages: early inflammation, progressive sclerosis and atrophy. The active stage of the disease is characterized by erythema ('lilac ring'), skin induration, and appearance of new or expansion of pre-existing skin lesions during the preceding month. The most recent localized scleroderma classification recognizes five types of the disease: localized, generalized, linear, deep and mixed. Various methods have been proposed for the evaluation of disease activity or severity and tissue damage, including ultrasonography, thermography, durometry, computed tomography and magnetic resonance imaging. However, all of them have their limitations, as they only make it possible to assess one component of the disease: activity or severity or tissue damage. The LS Cutaneous Assessment Tool (LoSCAT) questionnaire is a promising method of patient evaluation because of its availability, repeatability of results and sensitivity to changes resulting from treatment. STRESZCZENIETwardzina ograniczona jest rzadką chorobą autoimmunologiczną, która przebiega z zajęciem skóry, tkanki podskórnej i tkanek głębiej położonych. Choroba klinicznie ma trzy fazy: wczesną zapalną, postępującego stwardnienia i zanikową (atroficzną). Wykładnikami aktywnego procesu chorobowego są: rumień (lilac ring), stwardnienie skóry, pojawianie się nowych wykwitów skórnych lub powiększenie wcześniej istniejących zmian w ciągu ostatniego miesiąca. Najnowsza klasyfikacja wyróżnia pięć typów twardziny ograniczonej: ograniczona, uogólniona, linijna, głęboka i mieszana. Zaproponowano wiele metod oceny aktywności lub nasilenia choroby oraz uszkodzenia tkanek, takich jak ultrasonografia, termografia, durometria, tomografia komputerowa czy rezonans magnetyczny. Ograniczenia powyż-szych badań wynikają z możliwości oceny tylko jednego wykładnika -aktywności lub nasilenia choroby albo uszkodzenia tkanek. Przedstawiona metoda ewaluacji pacjentów oparta na zastosowaniu kwestionariusza do oceny aktywności i nasilenia choroby (LoSCAT) jest godna uwagi ze względu na dostępność narzędzia, powtarzalność wyników, a także czułość formularza na zmiany wynikające z zastosowanego leczenia.

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“…Bu nedenle, biz de bu çalışmada morfea lezyonlarının klinik takibinde düşük frekanslı USG tekniklerinin rolünü araştırmak için histopatolojik olarak morfea tanısı almış olan olgularda lezyonlu deri kalınlığı, elastisitesi, ekojenitesi ve vaskülaritesi gibi temel parametreleri kullanarak USG tekniği ile morfea hastalık aktivitesi, klinik tipleri, klinik değerlendirmeler arasındaki korelasyonun incelenmesini amaçladık (4,5).…”

Section: Introductionunclassified

Low Frequency Ultrasonography in Plaque-Type Morphea

Balevi¹,

Eren²,

Üstüner³

et al. 2017

tdd

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Amaç: Düşük frekanslı ultrasonografik incelemenin lokalize sklerodermada deri tabakalarını ve dokuların perfüzyon paternlerini belirleyen başarılı bir yardımcı teknik olduğu düşünülmektedir. Histopatolojik olarak morfea tanısı olan olgularda lezyonlu derinin kalınlığı, elastisitesi, ekojenitesi ve vaskülaritesi gibi temel parametrelere bakılarak düşük frekanslı ultrason tekniğinin klinik takipteki rolünün belirlenmesi amaçlanmıştır. Objective: Low frequency ultrasonographic examination is thought to be a successful adjuvant technique for determination of skin layers and tissue perfusion patterns in localized scleroderma. We aimed to identify the role of this technique in the clinical follow-up via investigation of basic parameters such as skin thickness, elasticity, echogenicity and vascularity in patients diagnosed with morphea by histopathology. Methods: Thirty-five plaque lesions of nineteen patients with morphea and symmetric, uninvolved cutaneous areas were compared by ultrasound in terms of mean skin thickness, elasticity index and elasticity ratio. The associations between the skin thickness and thickness score, elasticity score and elasticity index, echogenicity score and vascularity score were investigated in all tissue levels. Results: Of the morphea lesions, while the mean dermal skin thickness and dermal elasticity ratio were decreased compared to normal skin, the dermal vascularity was solely increased with an increase of the tissue thickness. Only the mean skin thickness was found to be significantly lower compared to the normal skin areas in the hypodermis of the morphea plaque lesions. Conclusion: Low frequency ultrasound can be used as an effective, adjuvant technique in the clinical follow-up of morphea plaque lesions in the levels of dermis and hypodermis.

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The Localized Scleroderma Skin Severity Index and Physician Global Assessment of Disease Activity: A Work in Progress Toward Development of Localized Scleroderma Outcome Measures

Cited by 162 publications

References 43 publications

Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases

Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases

Localized scleroderma – classification and tools used for the evaluation of tissue damage and disease activity/severity

Low Frequency Ultrasonography in Plaque-Type Morphea

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