Search

Background: Systemic sclerosis (SSc) patients often become refractory to proton pump inhibitors (PPI)—a standard treatment for gastroesophageal reflux disease (GERD)—and intolerant to PPI in combination with domperidone. PPI with alginic acid is an alternative treatment option, but alginic acid is costly. Objectives: We compared the costs and effectiveness of alginic acid plus PPI versus standard treatments (PPI with/without antacids as needed and lifestyle modifications) for GERD in SSc patients unsuitable for, or intolerant to, domperidone. Methods: An economic evaluation using the Markov model was conducted among SSc patients aged between 40 and 65 years with GERD, having a partial or non‐response to 4 weeks of standard‐dose omeprazole (40 mg/day) and being unsuitable for or intolerant to domperidone. Using a societal perspective, we computed the incremental cost‐effectiveness ratios (ICERs) in terms of Thai baht (THB) per quality‐adjusted life‐year (QALY) between a combination of alginic acid plus PPI and standard treatment for GERD. The lifetime time horizon was used. Results: The ICER for alginic acid plus PPI versus standard treatments was 377 101 THB/QALY. According to the one‐way sensitivity analysis, the cost of alginic acid was the most impactful parameter. If the market prices of alginic acid plus PPI were reduced by 61%, this treatment option would become cost‐effective at the willingness‐to‐pay threshold of 160 000 THB/QALY (34.68 THB/USD data on 25 May 2023). Furthermore, if alginic acid were included in the public health insurance program, the national budget would be increased by 66 313 THB per patient, resulting in an overall budget increase of 5 106 101 to 8 885 942 THB compared with the standard treatment. Conclusions: Alginic acid plus PPI does not represent good value for money compared with the standard treatment among such SSc patients in Thailand unless its price is reduced significantly. [ABSTRACT FROM AUTHOR]

Background: A better understanding of the epidemiological profile of systemic sclerosis (SSc) in Thais could improve care, human resource deployment, and public health budgeting. Objectives: We aimed to determine the incidence and prevalence of SSc in Thailand between 2017 and 2020. Methods: A descriptive epidemiological study was performed using the Information and Communication Technology Center, Ministry of Public Health database, comprising all types of healthcare providers during the study period. Demographic data of patients having a primary diagnosis of M34 systemic sclerosis and over 18 years of age between 2017 and 2020 were reviewed. The incidence and prevalence of SSc were calculated as well as their respective 95% confidence intervals (CIs). Results: The number of SSc cases in 2017 was 15,920 from a total Thai population of 65,204,797. The prevalence of SSc in 2017 was 24.4 per 100,000 populations (95% CI 24.0–24.8). The prevalence of SSc among women was 2 times greater than among men (32.7 vs. 15.8 per 100,000). The incidence of SSc was stable from 2018 to 2019 but dropped slightly in 2020 (7.2, 7.6, and 6.8 per 100,000 person-years, respectively). Most SSc cases were in northeastern Thailand (11.6, 12.1, and 11.1 per 100,000 person-years from 2018 to 2020, respectively) and the peak was between 60 and 69 years of age (24.6, 23.8, and 20.9 per 100,000 person-years from 2018 to 2020, respectively). Conclusion: SSc is a rare disease among Thais. The disease was commonly revealed in late middle-aged women with a peak at age 60–69 years, mainly from the northeast regions. The incidence remained stable during the study period, albeit during the emergence of the coronavirus pandemic a slight decline was recorded. Key Points • The incidence and prevalence of systemic sclerosis (SSc) vary across ethnic populations. • There is a lack of epidemiology research of SSc since the 2013 ACR/EULAR Classification Criteria for Scleroderma was adopted among Thais included Asia-Pacific population as the population has some different clinical features than those reported among Caucasians. • SSc is a rare connective disease among Thais and commonly presents in late middle-aged group of both genders, mainly in Thailand's northern and northeastern regions. • When compare to the epidemiology of SSc in Asia-Pacific, the prevalence of SSc in Thais was higher than among East Asians and the Indian population and the incidence of SSc among Thais was greater than the other Asia-Pacific population including Australian. [ABSTRACT FROM AUTHOR]

Available data including the incidence, predictors and long-term outcome of early systemic sclerosis patients associated with suspected cardiomyopathy(SSc-CM) is limited. Therefore, we aimed to study the incidence, predictors and survival of SSc-CM. An inception cohort study was conducted for early SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, Thailand, from January 2010 to December 2019. All patients were determined for clinical manifestations and underwent echocardiography and HRCT at enrollment and then annually. SSc-CM was determined and classified using echocardiography. 135 early SSc patients (82 female,108 DcSSc) were enrolled. With the mean follow-up period of 6.4 years, 32 patients developed SSc-CM. The incidence of SSc-CM was 5.3 per 100-person years. The multivariate Cox regression analysis showed that baseline anti-topoisomerase I-positive (Hazard ratio[HR] 4.86, p = 0.036), dysphagia (HR 3.35, p = 0.001), CK level ≥ 500 U/L(HR 2.27, p = 0.045) and low oxygen saturation (HR 0.82, p = 0.005) were predictors of SSc-CM. The survival rates after SSc-CM diagnosis at 1, 5 and 10 years were 90.3%, 73.1%, and 56.1%, respectively. In this study cohort, the incidence of SSc-CM was 5.3 per 100-person years, and tended to have low survival. The presence of anti-topoisomerase I antibody, dysphagia, CK level ≥ 500 U/L, and low oxygen saturation were independent baseline predictors for developing SSc-CM. [ABSTRACT FROM AUTHOR]

Background: Skin tightness is a hallmark of systemic sclerosis (SSc), and the fingers are an affected body part, so much so that fingerprints can be significantly affected among those with extensive skin tightness of the finger. Objective: We aimed to compare the difference between the current and past (pre-disease onset) fingerprints of SSc patients. Methods: We conducted a cross-sectional study among adult SSc patients who attended the Scleroderma Clinic, Khon Kaen University, between October 2019, and September 2020. All eligible patients consented to obtaining their current and previous prints from the Central Registration Bureau, Department of Provincial Administration, Ministry of the Interior. The current prints were obtained using the Crossmatch (Lite UE) live scan from the Central Institute of Forensic Science of Thailand. We investigated the concordance between the prints before disease onset and the current (enroll date) via the Printquest AFIS system with officers from the Central Institute of Forensic Science, Thailand. Results: One hundred SSc patients, according to the sample size calculation, were enrolled (mean age 59.4 ± 9.6 years; 66% female). Most (70%) had the diffuse cutaneous SSc subset. A respective 59%, 55%, and 6% presented acro-osteolysis, hand deformities, and digital ulcers. Some challenges were experienced in obtaining prints from patients with loss of the fingertip fat pad, finger joint contracture, and/or acro-osteolysis; notwithstanding, all fingerprints were usable and without individualized changes. Conclusion: Fingerprints were affected by fingertip lesions and finger joint contractures; notwithstanding, the prints remained usable for personal identification. Key Points • Skin involvement in systemic sclerosis (SSc) affects the prints, particularly at the fingertip. • Despite disease onset, the fingerprints of SSc patients do not change significantly. • Fingerprints are inadequate for personal identification among SSc patients with hand deformities due to poor quality or difficulty acquiring them. [ABSTRACT FROM AUTHOR]

Patients with systemic sclerosis (SSc) have some potential factors associated with an increased risk of sarcopenia. There has been currently no study to examine such associations in SSc patients. We aimed to determine the prevalence of sarcopenia among SSc patients and examine its association with clinical manifestations and laboratory tests. A cross-sectional study was conducted in 180 adult SSc patients at the Scleroderma Clinic, Khon Kaen University, Thailand, between July 2019 and April 2020. Clinical data, laboratory tests for inflammatory markers, serology, hormone, body composition by dual-energy X-ray absorptiometry, handgrip strength, functional lower extremity strength, and usual gait speed were collected and measured. Sarcopenia was defined according to the criteria of the Asian Working Group for Sarcopenia. One hundred and eighty patients were recruited. Ninety-four cases (52.2%) were the diffuse cutaneous SSc subset. The respective mean age and duration of disease was 58.8 ± 9.4 and 6.2 ± 5.3 years. Sarcopenia was revealed in 41 SSc patients for a prevalence of 22.8% (95% CI 12.1–34.8), while the prevalence was higher in patients with the diffuse cutaneous SSc (dcSSc) compared to the limited cutaneous SSc. BMI at the onset of SSc and C-reactive protein > 5 mg/dL were significantly associated with sarcopenia with a respective OR of 0.60 (95% CI 0.48–0.75) and 3.18 (1.06–9.54). Sarcopenia is common in patients with SSc, but the prevalence is more pronounced in the dcSSc. Inflammatory markers, particularly the CRP level, are strongly associated. BMI at the onset had a negative association with sarcopenia among SSc patients. [ABSTRACT FROM AUTHOR]

Clinical predictors of mortality in systemic sclerosis (SSc) are diversely reported due to different healthcare conditions and populations. A simple predictive model for early mortality among patients with SSc is needed as a precise referral tool for general practitioners. We aimed to develop and validate a simple predictive model for predicting mortality among patients with SSc. Prognostic research with a historical cohort study design was conducted between January 1, 2013, and December 31, 2020, in adult SSc patients attending the Scleroderma Clinic at a university hospital in Thailand. The data were extracted from the Scleroderma Registry Database. Early mortality was defined as dying within 5 years after the onset of SSc. Deep learning algorithms with Adam optimizer and different machine learning algorithms (including Logistic Regression, Decision tree, AdaBoost, Random Forest, Gradient Boosting, XGBoost, and Autoencoder neural network) were used to classify SSc mortality. In addition, the model's performance was evaluated using the area under the receiver operating characteristic curve (auROC) and its 95% confidence interval (CI) and values in the confusion matrix. The predictive model development included 528 SSc patients, 343 (65.0%) were females and 374 (70.8%) had dcSSc. Ninety-five died within 5 years after disease onset. The final 2 models with the highest predictive performance comprise the modified Rodnan skin score (mRSS) and the WHO-FC ≥ II for Model 1 and mRSS and WHO-FC ≥ III for Model 2. Model 1 provided the highest predictive performance, followed by Model 2. After internal validation, the accuracy and auROC were good. The specificity was high in Models 1 and 2 (84.8%, 89.8%, and 98.8% in model 1 vs. 84.8%, 85.6%, and 98.8% in model 2). This simplified machine learning model for predicting early mortality among patients with SSc could guide early referrals to specialists and help rheumatologists with close monitoring and management planning. External validation across multi-SSc clinics should be considered for further study. [ABSTRACT FROM AUTHOR]

Objective Lower urinary tract symptoms (LUTS) are common in SSc. The severity of symptoms can affect the quality of life (QOL); however, LUTS is often neglected during routine assessments. We determined the prevalence of moderate to severe LUTS in SSc and its associated factors. Methods A cross-sectional study was conducted between March 2020 and June 2020. Adult SSc patients were enrolled from the Scleroderma Clinic, Khon Kaen University, Thailand. All completed a self-administered questionnaire on LUTS using the International Prostate Symptom Score (IPSS), categorized into absent, mild, moderate or severe LUTS. In addition, we investigated the factors associated with moderate to severe LUTS, and the correlation between IPSS-QOL score and IPSS severity. Results A total of 135 patients were enrolled. Most cases were female (87 cases; 64.4%) and had dcSSc (88 cases; 65.2%). Twenty-six were defined as having moderate to severe LUTS, for a prevalence of 19.3% (95% CI 13.0, 26.9%). In addition, most had storage symptoms (63.0%), followed by voiding symptoms (19.3%) and post-voiding symptoms (12.6%). The factors associated with moderate to severe LUTS per the multivariable logistic regression included a modified Rodnan skin score ≥20 points and gastrointestinal symptoms with adjusted odds ratios 7.64 and 5.78, respectively. In addition, the IPSS-QOL score had a moderate positive correlation with IPSS severity (rho  =   0.560, P <  0.001). Conclusion Moderate to severe LUTS occurred in about one-fifth of SSc patients, particularly those with extensive skin tightness and gastrointestinal involvement. The more severe the LUTS, the poorer the QOL. [ABSTRACT FROM AUTHOR]

Background : Inception cohort data regarding the incidence of cardiopulmonary complications in early systemic sclerosis (SSc) patients comparing those with and without elevated baseline creatine kinase (CK) are limited. This study aimed to compare the incidence of cardiopulmonary complications and survival between the two subgroups. Methods: We used an inception cohort study of early SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, Thailand, from January 2010 to December 2019. All patients were assessed for clinical manifestations and CK levels and underwent echocardiography and HRCT at the study entry and annually thereafter. Results: A total of 144 SSc patients (84 female, 115 diffuse cutaneous SSc (DcSSc)) with a mean disease duration of 11.9 ± 9.2 months were enrolled. At cohort entry, their mean ± SD CK levels were 364.3 ± 598.0 U/L. The participants were then divided into two subgroups: (i) 29 SSc with elevated CK (baseline CK ≥ 500 U/L); (ii) 115 SSc with non-elevated CK. At enrollment, the elevated CK group was characterized by a higher proportion of male gender, DcSSc subtype, arthritis, and weakness; shorter disease duration; and higher MRSS compared with non-elevated CK. At the last visit, with a mean ± SD follow-up duration of 6.2 ± 2.7 years, the elevated CK group showed a higher cumulative prevalence of weakness, dysphagia, LVEF < 50%, and suspected myocardial disease; higher incidence of LVEF < 50%, suspected myocardial disease, and ILD; and shorter survival time. Conclusion: It was found in our study cohort that elevated baseline serum CK in early SSc, of which majority were DcSSc subtype, is associated with more severe clinical presentation, higher incidence of cardiopulmonary complications, and shorter survival time compared with the non-elevated CK subgroup. Key Points • In early SSc patients, elevated baseline serum creatine kinase was confirmed to be associated with a high incidence of cardiac and ILD complications, and poor long-term survival time. • Careful evaluation of baseline serum CK levels in all early-diagnosed SSc patients is crucial in general clinical practice. [ABSTRACT FROM AUTHOR]

Objectives: This study is aimed at determining the role of T cells by assessing the numbers of IFN-γ- and IL-2-secreting T cells following stimulation with peptides derived from DNA topoisomerase-I protein in Thai SSc patients.Methods: Fifty Thai SSc patients and 50 healthy controls (HC) joined this study. IFN-γ and IL-2 levels upon stimulation of T cells with 6 peptides derived from DNA topoisomerase-I protein were determined. Anti-nuclear antibodies (ANA) and anti-Scl-70 antibodies were determined by using the ELISA method.Results: In SSc patients, we detected a significantly higher number of IFN-γ- and IL-2-secreting CD8+ T cells than IFN-γ- and IL-2-secreting CD4+ T cells after stimulation with pooled peptides derived from DNA topoisomerase-I protein. A similar percentage of CD4+IL-2+, CD4+IFN-γ+, and CD8+IL-2+ were detected following stimulation with DNA topoisomerase-I protein -in SSc patients with anti-Scl-70 antibody (SSc/anti-Scl-70+) and those without. In contrast, the amount of CD8+IFN-γ+ cells was significantly higher in SSc/anti-Scl-70+ than those without. Stimulation with individual peptides showed that CSLRVEHINLHPELD (sPep3; 15 amino acids; position 505-519 of DNA topoisomerase-I protein) was the optimal epitope that induced T cells secreting the highest levels of IFN-γ and IL-2. A higher percentage of IFN-γ+CD4+ T cells was detected in SSc/anti-Scl-70+ than those without the following stimulation with peptides 2 (amino acid position 475-486 [RAVALYFIDKLA] of protein DNA topoisomerase).Conclusion: The results from this study emphasize the critical role of DNA topoisomerase-I peptides on the activation of T cells in SSc patients. The findings provide a better understanding of SSc's immunopathogenesis and may lead to the development of diagnostic tools and specific treatments for SSc in the future. [ABSTRACT FROM AUTHOR]

Scleroderma, particularly systemic sclerosis, is a chronic illness that affects a person's physical, emotional, psychosocial, and spiritual dimensions. To investigate self-care deficits, health behaviours, and trajectory management of Thai people with systemic sclerosis, an exploratory case study method was used through interviews and patient records. Self-care deficit nursing theory and the chronic illness trajectory framework guided the research. Twelve patients with systemic sclerosis were purposively recruited at a hospital clinic in the northeastern region of Thailand. Thai people living with systemic sclerosis developed specific self-care agencies in each of 3 identified trajectory patterns to improve their health and well-being. By applying the theory and framework that guided the research, nurses can promote self-care behaviours and recognize situations along an illness trajectory that require intervention and management. The results extend the understanding of how people live and cope with systemic sclerosis. [ABSTRACT FROM AUTHOR]

Introduction: Systemic sclerosis is a systemic connective tissue disease with variable cutaneous presentations. Although pigmentary disturbances have been described in systemic sclerosis, a reticulate hyperpigmentation has only been reported in one case of systemic sclerosis to date.Case Presentation: We describe a previously healthy 51-year-old Thai woman who presented with a reticulate hyperpigmentation affecting her trunk and extremities, together with sclerodactyly and proximal sclerosis, resulting in a new diagnosis of systemic sclerosis.Conclusions: To date, the pathogenesis of reticulate hyperpigmentation in systemic sclerosis remains unclear. Increased melanin synthesis and altered thermoregulatory mechanism are proposed to be involved in the pathogenesis of this presentation. This case represents an unusual cutaneous feature of reticulate hyperpigmentation in the setting of systemic sclerosis. [ABSTRACT FROM AUTHOR]

Objective. To determine the prevalence of asymptomatic cardiac involvement and its correlation with non-cardiac manifestation in Thai SSc patients. Methods. A cross-sectional study was carried out between January 2012 and June 2013 at Srinagarind Hospital, Khon Kaen University, Thailand, on adult SSc patients without signs or symptoms suggestive of cardiac involvement. We excluded those with overlap syndrome, having serum creatinine >123.8 µmol/l, history of cardiac diseases, any atherosclerosis risk factors and receiving angiotensin-converting enzyme inhibitors. Non-invasive tests related to cardiac involvement were performed, including: echocardiography, ECG, chest X-ray, inflammatory biomarkers, cardiac enzymes and N-terminal prohormone of brain natri-uretic peptide. Results. A total of 103 SSc patients were enrolled, 61.2% of whom were in the subset. Of these, 63 patients had at least one test abnormality (prevalence 61.2%; 95% CI 51.6, 70.7). The two leading cardiac abnormalities were diastolic dysfunction (44.7%) and elevation of cardiac enzymes (36.9%). The only predictor for cardiac involvement per multivariate analysis was the dcSSc subtype with a higher modified Rodnan skin score, and shorter disease duration (odds ratio = 3.37; 95% CI 1.07, 10.65). Compared with the limited subtype, dcSSc was also significantly associated with elevated cardiac enzyme and prolonged distance between a Q wave and T wave in an ECG (QT interval). Conclusion. Asymptomatic cardiac involvement in Thai SSc was not uncommon, and the most common finding was diastolic dysfunction. Elevated cardiac enzymes were found in one-third of the patients, which correlated with the dcSSc subtype with a higher modified Rodnan skin score and shorter disease duration, suggestive of early myocardial microcirculation disruption. Long-term follow-up was performed to elucidate the clinical implication of these abnormalities. [ABSTRACT FROM AUTHOR]

This study aimed to identify the indications for hospitalization, hospital mortality rate, predictors of hospital mortality, and clinical parameters affecting length of stay (LOS) among Thai systemic sclerosis (SSc). A retrospective study was performed in SSc patients admitted in Khon Kaen University, Thailand, between January 2008 and December 2010. The respective clinical factors affecting LOS and predictors of mortality were analyzed using the Spearman's rank correlation and the Cox regression model. There were 202 hospital admissions among 131 SSc patients. The female-to-male ratio was 1.6:1. The median age at admission was 54.7 years (interquartile range (IQR) 49.2-62.9), the duration of disease at admission was 2.9 years (IQR 1.1-7.8), and the LOS was 5 days (IQR 2-10). The indications for hospitalization were divided equally between SSc-related and non-SSc-related events (53.5 vs. 46.5 %, respectively). The most common indication for hospitalization was infection (23.3 %) and pneumonia is the most common cause of infection (58.0 %). Prolonged LOS was related to fatigability status ( p < 0.01), intestinal involvement ( p < 0.01), electrolyte disorders ( p < 0.01), multiple comorbidities ( p < 0.01), modified Rodnan skin score ≥20 points ( p = 0.01), disease duration under 5 years ( p = 0.02), cardiac arrhythmia ( p = 0.04), and deficiency anemia ( p = 0.04). Hospital mortality was 16.8 per 100 person-years (95 % confidence interval (95 % CI) 10.8-24.3). Infection (59.1 %) was the most common cause of death, particularly from bacterial pneumonia. Clinical predictors of mortality were: disseminated intravascular coagulation related to infection (hazard ratio (HR) 52.73; 95 % CI 1.26-403.74), cardiac arrhythmia (HR 32.89; 95 % CI 3.00-359.95), electrolyte disorders (HR 15.66; 95 % CI 2.04-119.98), renal crisis (HR 13.38; 95 % CI 1.80-99.36), intestinal involvement (HR 10.42; 95 % CI 2.58-42.01), admission due to a non-SSc-related condition (HR 8.93; 95 % CI 2.21-36.13), and disease duration under 5 years (HR 6.67; 95 % CI 1.21-36.52). Infection was the most common cause of hospitalization. Prolonged LOS and hospital mortality should be warning signs in patients with shorter disease duration, presence of intestinal involvement, cardiac arrhythmia, and multiple comorbidities. [ABSTRACT FROM AUTHOR]

Background Reports of hospitalized systemic connective tissue disorders ( SCNTD) are mostly disease-specific reports from institutional databases. Objective To clarify the admission rate, disease determination, hospital mortality rate, length of stay and hospital charges among hospitalized patients diagnosed with SCNTD. Materials and methods The data were extracted from the 2010 national database of hospitalized patients provided by the Thai Health Coding Center, Bureau of Policy and Strategy, Ministry of Public Health, Thailand. Patients over 18 years having International Classification of Diseases ( ICD)-10 codes for a primary diagnosis related to SCNTD were included. Results There were 6861 admissions coded as disorders related to SCNTD during the fiscal year 2010. The admission rate was 141 per 100 000 admissions. Among these, systemic lupus erythematosus ( SLE) was the most common, followed by systemic sclerosis ( SSc) and dermatomyositis/polymyositis ( DM- PM). The overall mean length of hospital stay was 6.8 days. Small vessel vasculitis and Sjögren syndrome had the longest and the shortest hospital stays respectively (14.5 vs. 5.3 days). Hospital charges were highest among systemic vasculitis and DM- PM patients. Conclusion The admission rate for SCNTD in Thailand was 141 per 100 000 admissions among which SLE was the most common. Overall hospital mortality was 4.1%. Although a lower prevalence was found among systemic vasculitis, it had a higher mortality rate, longer length of stay and greater therapeutic cost. [ABSTRACT FROM AUTHOR]

Background Infection is a common cause of death in systemic sclerosis ( SSc) but despite immunosuppressant therapy, there are few reports of opportunistic infection. Objectives To estimate the incidence of infection, to determine the etiologic organism, and to assess the risk factors for infection among Thai SSc patients. Methods A historical cohort analysis was conducted on patients over 15 years of age, diagnosed with SSc, who attended the Scleroderma Clinic at Srinagarind Hospital, Khon Kaen, Thailand, between January 1, 2005 and December 31, 2006. Results The medical records of 117 SSc patients were reviewed. The female-to-male ratio was 1.5 : 1.0. Of the total 310 person-years under observation, 63 events of infection occurred. The incidence rate of infection was 20.3 per 100 person-years (95% CI 15.6-26.0) and the incidence rate of major infection was 11.0 per 100 person-years (95% CI 8.4-16.5). The mean age and mean duration of SSc at the time of infection was 50.1 ± 11.1 years (range, 25.2-76.6) and 12.9 ± 10.4 months (range, 0.5-34.6), respectively. Urinary tract infection was the most common infection (23.8%). Opportunistic infection was found in one case (esophageal candidiasis). Esophageal dysmotility was significantly related to major infection (odds ratio [ OR] 3.22). There was a clinical association between aspiration pneumonia and esophageal dysmotility ( OR 1.23), as well as non-strongyloidiasis diarrhea and gastrointestinal involvement ( OR 2.28). One person died due to severe bacterial aspiration pneumonia. Conclusions Infection is not uncommon among SSc patients; however, opportunistic infection is rare, despite immunosuppressant therapy. Esophageal dysmotility increases the risk of major infection, particularly of aspiration pneumonia. [ABSTRACT FROM AUTHOR]

Background: Systemic sclerosis (SSc) is a multisystem disorder that affects various organ systems. Although SSc patients have both physical and psychological illness, psychological distress is sometimes underestimated as most physicians usually pay more attention to physical problems. Aims: To evaluate dermatology-specific health-related quality of life (QoL) in Thai patients with SSc. Methods: SSc patients, who attended the Department of Dermatology, Siriraj Hospital, Bangkok, Thailand, between August 2009 and April 2010, were enrolled. The demographic data and skin manifestations of the patients were recorded. Skin thickness of each patient was analyzed by modified Rodnan skin score. QoL was evaluated by using the validated Thai version of dermatology life quality index (DLQI) questionnaire. Results: A total of 80 patients of SSc were enrolled in this study. Twelve patients had limited SSc, while 68 patients had diffuse SSc. The mean (SD) disease duration before period of evaluation was 9 (12.8) years. The mean total of DLQI score was 6.3 (range, 0-23). Patients with low DLQI score have longer disease duration than patients with high DLQI score (P<0.05). Pain/pruritus was the most significant problem to the patients. Salt and pepper appearance was the cutaneous finding that had association with high DLQI score. Conclusions: SSc had moderate impact on patient's dermatology-specific health-related QoL. Pain, pruritus, and obvious skin findings caused a significant impairment to SSc patients. Therefore, the treatment of pain and pruritus and prominent cutaneous findings should be taken into account to improve QoL of SSc patients. [ABSTRACT FROM AUTHOR]

Aims: Pulmonary arterial hypertension (PAH) is a major complication and cause of death in systemic sclerosis (SSc). Our objective was to identify the predictive factors of mortality and the 2-year survival rate among Thai sufferers of PAH-SSc. Methods: An historical cohort study was performed among PAH-SSc patients followed up at Srinagarind Hospital, Thailand, between January 2005 and December 2008. Kaplan-Meier and Cox regression analyses were used to estimate the probability of survival and to assess the significant factors associated with death. Results: Pulmonary arterial hypertension was recognized in 60 patients using ECHO criteria, right ventricular systolic pressure (RVSP) > 35 mmHg. Two-thirds of the patients were female, > 50 years of age, with the dSSc subtype. Twenty patients (33.3%) died: the mortality rate was 15.6% per 100 person-years. The respective 1-, 2-, 3- and 4-year survival rate was 86.1%, 71.3%, 64.6% and 53.9%. The majority (85%) died without any specific treatment for PAH. Using univariate analysis, the mortality risk was associated with: the WHO functional class (FC) III (HR = 27.82), visceral organ involvement (HR = 5.14), myositis (HR = 3.14), esophageal dysmotility (HR = 3.08) and pericarditis (HR = 2.84). Using Cox regression, the only predictor of death was FCIII. The causes of death in PAH-SSc were related to PAH (60%), infection (30%) and acute renal failure (10%). Conclusion: Up to one-third of Thai sufferers of PAH-SSc died within 2 years of PAH diagnosis, without any specific treatment being given. Increased mortality risk was found in SSc patients who had FCIII and visceral organ involvement. [ABSTRACT FROM AUTHOR]