Correlation between the infrared thermogram and microvascular abnormalities of the nailfold in patients with systemic sclerosis.

INTRODUCTION: Systemic sclerosis (SSc) is a multisystemic disease with an extensive microvasculopathy. The gold standard for its investigation is nailfold videocapillaroscopy (NVC). AIM: To assess the value of thermography (IRT) for the assessment of microvasculopathy in patients with SSc. MATERIAL AND METHODS: Nineteen patients with limited cutaneous SSc were enrolled in the study. They underwent IRT imaging and NVC. An average temperature (Tavg) at the nailfold and a gradient of temperatures (ΔTavg) between the central metacarpus of the hand and the nailfold was determined for all fingers. NVC pictures were classified to capillaroscopic patterns according to Cutolo et al. system and they were analysed quantitatively to measure the density of capillaries and to calculate capillaroscopic skin ulcers risk index (CSURI) for each finger separately. RESULTS: There was only a moderate correlation (0.4 < r < 0.6) between thermographic parameters and density of capillaries in fingers II-V (r = 0.5; p < 0.001 for Tavg and r = -0.45; p < 0.001 for ΔTavg), but none in thumbs (r = 0.29; p = 0.089 for Tavg and r = -0.19; p = 0.275 for ΔTavg). Early pattern was associated with a significantly greater surface temperature (Tavg) of nailfolds and essentially milder ΔTavg in fingers II-V when compared to all other capillaroscopic patterns in fingers II-V. Surface temperature (Tavg) was significantly lower and ΔTavg was markedly more pronounced in fingers II-V with a greater risk of development of digital ulcers (DU) calculated by CSURI. CONCLUSIONS: Although IRT measurements correlate only moderately with density of capillaries, this technique seems to be substantial to determine the capillaroscopic pattern and to identify patients at greater risk of DU development.

Health-related quality of life and its influencing factors in adult patients with localized scleroderma - a cross-sectional study.

BACKGROUND: Localized Scleroderma (LoS) is an autoimmune connective tissue disease that affects skin and less commonly subcutaneous tissues. The illness occurs in children and adults, and may have a serious impact on health-related quality of life (HRQoL). The goal of this study was to explore what factors might deteriorate scores on HRQoL measures in adult LoS patients. METHODS: Detailed information on the demographic and clinical features of LoS patients was collected. The HRQoL was assessed using Skindex-29 and Short form 36 (SF-36) questionnaires. RESULTS: Thirty three women and seven men with LoS were enrolled. Female gender negatively influenced scores for the emotion subscale of Skindex-29. Multiple-factor linear regression analysis confirmed, as with single-factor analysis, that the causes of low SF-36 physical component score (PCS) were subjective symptoms (pruritus, pain, paresthesia), musculoskeletal manifestations and older age at the time of survey, while rural area of residence negatively affected the SF-36 mental component score. Additionally, single-factor analysis showed that the SF-36 PCS was related to the LoS cutaneous assessment tool (LoSCAT) summary score. CONCLUSIONS: Apart from a clinical presentations, sociodemographic characteristics, including older age, female gender and living conditions, may impair HRQoL of LoS patients. Further studies that will examine the role of these factors for physical and mental functioning of adults with LoS are needed.

Associations between Disease Activity/Severity and Damage and Health-Related Quality of Life in Adult Patients with Localized Scleroderma-A Comparison of LoSCAT and Visual Analogue Scales.

Localized scleroderma (LoS) is a chronic fibrosing disorder of the skin and, less commonly, subcutaneous tissues. As the disease causes subjective symptoms, cosmetic defects, and, at times, functional disability, subjects with LoS experience deterioration of their health-related quality of life (HRQoL). The influence of disease activity/severity and damage status on HRQoL measures in patients with LoS is scarcely known. Physician-reported measures (modified LoS skin severity index, LoS skin damage index, physician global assessments of the disease activity/severity and damage) and patient-derived measures (patient global assessments of the disease activity/severity and damage) were obtained in adult LoS patients. Their HRQoL was measured with Skindex-29 and Short Form-36. The patients' assessments of disease activity/severity and damage in LoS differed from the assessments by the physicians. The patients' predominant concerns centered on LoS-related damage, whereas the physicians' concerns focused on features of disease activity. Visual analogue scales bore some relation to the HRQoL, and they seem to be important in a holistic approach to the patient and should not be omitted in LoS evaluation.

An extended high-frequency ultrasound protocol for assessing and quantifying of inflammation and fibrosis in localized scleroderma.

BACKGROUND: Clinical characteristics of the lesions are used to identify activity and damage in localized scleroderma (LoS). For high-frequency ultrasound (HF-US), the features of active lesions were described. MATERIALS AND METHODS: Clinical signs of activity and damage in LoS lesions were assessed with the use of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and HF-US by two examiners independently. All US images were obtained using a 20 MHz HF-US (DermaLab System, Cortex Technology, Hadsund, Denmark). The dermal thickness (DT) and echogenicity (intensity score, IS) of the LoS lesional dermis were measured in the area of each lesion with the highest score for erythema (ER), skin thickness (ST), and dermal atrophy (DAT). Measurements were compared to the site-matched unaffected skin. The relative difference of DT and IS values was calculated between each lesion and its normal control for comparison among different clinical scores for ER, ST, and DAT. RESULTS: A total of 92 lesions in 40 adult patients were examined with HF-US. Thirty one lesions were erythematous, 26 were in sclerosis, and 35 were in atrophy. A correlation between the clinical evaluation of the LoS lesions and US measurements was found. The sensitivity and specificity of HF-US were 97% and 90%, respectively. The positive predictive value was 83%, negative predictive value-98%. Interrater reliability was excellent for LoSCAT and HF-US findings. CONCLUSION: High-frequency ultrasound allows an accurate assessment of the inflammatory and fibrotic skin lesions in LoS.

Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) adapted for use in adult patients: report from an initial validation study.

BACKGROUND: Localized scleroderma (LoS) affects both children and adults and is associated with permanent functional and cosmetic impairment, and reduced quality of life predominating in adults. The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is a clinical instrument designed to measure an activity and damage of LoS. It has been validated for use with pediatric LoS patients. This study assessed the validity and reliability of the LoSCAT adapted for use in adults. METHODS: Before the initiation of the study two examiners participated in an intensive training course carried out by an expert in LoS. Appendices describing each LoSCAT domain were prepared. Features determining disease activity and damage in adult LoS patients were identified to properly evaluate the physician (Phys) and patient (Pt) global assessment (GA) of disease activity (A)/severity (S) and damage (D), which were used to assess convergent validity of the LoSCAT. Correlations of physician- and patient-derivied measures with Skindex-29 were also analysed. RESULTS: The study included 40 adult LoS patients (33 females and 7 males) with different subtypes of LoS. Intra and inter-rater reliability of the LoSCAT was found to be excellent. Positive correlations were observed between the PhysGA-A, PhysGA-D, PtGA-A and the LoSCAT's domains, while no correlations between them and the PtGA-D were found. There were no relationships between LoSCAT's components and Skindex-29. CONCLUSIONS: Despite the LoSCAT is a reliable tool for an assessment of cutaneous lesions, additional health status instruments are necessary to a holistic approach to LoS in adults.