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Axial spondyloarthritis (axSpA) increases the risk of osteoporosis and vertebral fractures. Bone mineral density (BMD) measured by dual X-ray absorptiometry (DXA) has limitations in axSpA patients. Trabecular bone score (TBS) indirectly assesses bone microarchitecture and can be used to predict fracture risk. However, few studies have investigated the role of TBS in axSpA patients. The objective of this study were to compare TBS between axSpA patients and 1:1 sex- and age-matched healthy volunteers and determine factors associated with low TBS in axSpA patients. A cross-sectional study was conducted in two tertiary-care hospitals. A total of 137 axSpA patients and healthy volunteers were enrolled. Demographics, disease characteristics, and risk factors for osteoporosis were recorded. TBS, BMD at the lumbar spine, hip, and vertebral fractures were assessed by DXA. Low TBS was defined as a TBS value < 1.230. Factors associated with low TBS were examined by logistic regression. Most patients were male (75.9%) and tested positive for HLA-B27 (88.3%). The mean (SD) age was 42.8 (12.0) years. The mean (SD) of TBS in the axSpA patients was lower than those in the healthy volunteers [1.402 (0.107) vs 1.440 (0.086), respectively; p = 0.002]. The mean (SD) of lumbar BMD in the axSpA patients was higher than in healthy volunteers [1.186 (0.212) vs 1.087 (0.124), p < 0.001], whereas the mean (SD) of femoral neck BMD in the axSpA group was lower than that in the healthy volunteers [0.867 (0.136) vs 0.904 (0.155), p = 0.038]. Disease severity as indicated by sacroiliac joint fusion and a high ASDAS score were associated with low TBS with the odds ratios (95% confidence interval) of 11.8 (1.2–115.4) and 5.2 (1.6–16.9), respectively. In conclusion, axSpA patients had a higher prevalence of low TBS than healthy volunteers. Sacroiliac joint fusion and a high ASDAS score were associated with low TBS. [ABSTRACT FROM AUTHOR]

Background: Coronavirus disease‐2019 (COVID‐19) vaccine hesitancy is a significant threat to the success of COVID‐19 vaccination programs. Objectives: We aimed to assess attitudes and factors affecting the decision‐making vis‐à‐vis COVID‐19 vaccination among patients with autoimmune rheumatic diseases (ARDs). Method: A cross‐sectional survey of adults with ARDs was conducted between January 2022 and April 2022. All enrolled ARDs patients were asked to answer a questionnaire about their attitudes regarding COVID‐19 vaccination. Results: Three hundred patients were included with a female‐to‐male ratio of 2.5:1. The mean age of the patients was 49.2 ± 15.6 years. Around 37% of patients who hesitated to get the COVID‐19 vaccination were apprehensive regarding potential adverse events from the vaccine. About 25% (76 cases) were hesitant about vaccination, of which 15% were uncertain about the vaccine's efficacy, and 15% thought the vaccine was unnecessary because they lived in rural areas where they practiced social distancing. "Family role as a non‐working member" was the only factor strongly associated with hesitancy for vaccination (odds ratio of 2.42; 95% CI 1.06–5.57). The attitudes to vaccination showed that the patients were concerned about disease flaring and believed all medicine should be stopped before vaccination. Conclusion: Around one‐quarter of ARDs sufferers hesitated to get COVID‐19 vaccination. In addition, some patients were disinclined to get vaccinated because they were worried about its efficacy and/or associated adverse events. The findings help healthcare providers plan to counter negative attitudes toward vaccination in ARDs patients to protect them during the COVID‐19 era. [ABSTRACT FROM AUTHOR]

Background: Erectile dysfunction (ED) has been reported among patients with systemic sclerosis (SSc) and primarily limited cutaneous SSc in Caucasians. While there is no data on ED among Thais in whom the diffuse cutaneous SSc subset is common. Objectives: We aimed to estimate the prevalence of ED among Thais with SSc, evaluate its severity, and determine the associated factors. Methods: We did a cross-sectional study among adult Thai male SSc patients. All eligible patients: a) completed the IIEF-15 questionnaire by themselves; b) underwent a genital examination by an experienced urologist to evaluate skin tightness of the penis, scrotum, and phimosis; and, c) were evaluated for Erection Hardness Score. Results: A total of 60 male SSc patients were included. The respective mean age and median disease duration was 54.8±7.2 years and 3.1 years (IQR 1.2–7.2). The definition of ED was fulfilled in 53 cases for a prevalence of 88.3% (95%CI 77.4–95.2), while 65% had severe ED, and none had skin tightness of the genitalia. Eight cases had acquired phimosis, and all were in the ED group. The patients with ED vs. those without ED had significantly lower scores for orgasm, sexual desire, and intercourse satisfaction, and trended to be older, have more severe skin tightness and have higher BMI. Conclusion: ED is a common problem in men with SSc and is mainly categorized as severe. The severity of SSc might increase the risk of developing ED. We found phimosis was a common genital abnormality co-occurring with ED in SSc. [ABSTRACT FROM AUTHOR]

Introduction: Standard regimens for scleroderma interstitial lung diseases (SSc-ILDs) are pulse intravenous (IV) and oral daily cyclophosphamide (CYC). However, IV CYC has limited access to diffuse cutaneous SSc, and oral daily CYC is associated with febrile neutropenia and hemorrhagic cystitis. Pulse oral CYC regimen has never been studied. Objective: To determine the effectiveness of pulse oral CYC therapy in SSc-ILDs, predictors of effectiveness, and side-effects. Methods: A historical cohort study enrolled SSc-ILDs from the SSc database registry at Srinagarind Hospital, Thailand from 1 January 2012 to 1 October 2018. All patients received monthly oral dosages of CYC 600–750 mg/m2, Mesna, and daily prednisolone of 10 mg for 2 years. Changes of FVC, chest radiography, HRCT, 6MWT, and side effects were recorded for the baseline and at the end of the treatment. Response to treatment was defined by (a) stable FVC or a decline ≤ 10% of predicted, (b) unchanged or improved radiographic findings, or (c) a decline 6MWT of ≤ 30 m compared with the baseline. Results: A total of 76 patients with female 52 patients (68.4%) and with a median age of 54.2 years (IQR 46.6–59.6). The majority was dcSSc subset (59 patients; 78.6%). Fifty-four patients (71%) were defined as responsive to therapy. The mean FVC improvement was 1 ml (SD 9.5). The only factor associated with treatment response was limited cutaneous SSc (OR 7.69, 95% CI (1.01, 339.68), p = 0.029). Hemorrhagic cystitis was found in 1 patient. Conclusions: Nearly three-quarters of SSc-ILDs patients had a good response to the pulse oral CYC therapy for 2 years with a few serious side effects. Pulse oral CYC therapy had been effective for SSc-ILDs in case of difficult IV access. Key Point • Pulse oral cyclophosphamide has been used for scleroderma lung disease. It has shown efficacy and safety in scleroderma patients. In patients who have difficulty with intravenous access, pulse oral cyclophosphamide can be an alternative regimen. [ABSTRACT FROM AUTHOR]

Background Pyogenic arthritis ( PA) is still a problematic arthritic disease that requires hospitalization. Objective To study the epidemiological characteristics and predictors of treatment outcomes for Thai patients hospitalized with PA. Materials and methods The nationwide hospital database from the 2010 fiscal year was analyzed. Patients 18 years of age onward, who had primary diagnosis of pyogenic arthritis, were included in this study. Results There were a total of 6242 PA admissions during 2010. It was ranked third among hospitalized musculoskeletal patients after osteoarthritis ( OA) and gouty arthritis. The estimated prevalence of PA was 13.5 per 100 000 adult population. Geographic distributions of PA was related to the population density of each region; however it seemed more frequent in the northern and northeastern regions of Thailand. The prevalence increased with age, 3.6 and 43.6 per 100 000 in young adults and the elderly, respectively. Among the 2877 co-morbidities coded, diabetes was the most common, followed by crystal-induced arthritis, existing other foci of infections (urinary tract infection, skin and soft tissue infections and pneumonia) and pre-existing chronic joint diseases ( OA,rheumatoid arthritis), respectively. Overall hospital mortality rate was 2.6%. Poorer outcomes were found among patients with chronic liver disease and other existing foci of infections. Conclusions The prevalence of hospitalized PA is still modest in Thailand, showing the highest prevalence in the advanced age group. Diabetes was the most commonly co-morbidity found; however, poorer outcomes were noted among patients with chronic liver disease and existing multiple sites of infections. [ABSTRACT FROM AUTHOR]

Background Skin thickness progression in scleroderma ( SSc) varies in daily clinical practice observation. Objectives To define the pattern of skin thickness among Thai sufferers of SSc and to ascertain the clinical correlation with each skin pattern. Methods A 3-year follow-up cohort was performed on patients over 15 years of age in Khon Kaen, Thailand, between January 1, 2005 and December 31, 2006. The progression of skin thickness equals the modified Rodnan skin score at the end minus the score at onset divided by the duration of the disease. Results SSc cases (117) were included and the female-to-male ratio was 70 : 47. The mean age at onset was 49.8 years (range 24.4-75.5). The most common skin patterns were: (i) 'slow progression to peak then slow regression' (77 cases; 65.8%); followed by (ii) 'continuous slow progression' (37; 31.6%); (iii) 'continuous intermediate progression' (2; 1.7%); and (iv) 'slow progression to peak then intermediate regression' (1; 0.9%). The respective mean peak skin score and duration of disease at peak skin score was 19.8 points (range 4-45) and 20.3 months (range 1.0-42.2). Only telangiectasia at onset and contracture of joint(s) were related to 'continuous slow progression' of skin thickness with P = 0.001 and P = 0.042, respectively. Neither SSc subsets nor internal organ involvement were correlated with skin thickness pattern. Conclusion The most common skin pattern in Thai SSc was 'slow progression to peak then slow regression'. Telangiectasia at onset and contracture of joint(s) were predictive of continuous progressive skin thickness in the first 3 years. [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]

Skin tightness is a major clinical manifestation of systemic sclerosis (SSc). Importantly for both clinicians and patients, spontaneous regression of the fibrosis process has been documented. The purpose of this study is to identify the incidence and related clinical characteristics of spontaneous regression among Thai SSc patients. A historical cohort with 4 years of follow-up was performed among SSc patients over 15 years of age diagnosed with SSc between January 1, 2005 and December 31, 2006 in Khon Kaen, Thailand. The start date was the date of the first symptom and the end date was the date of the skin score ≤2. To estimate the respective probability of regression and to assess the associated factors, the Kaplan-Meier method and Cox regression analysis was used. One hundred seventeen cases of SSc were included with a female to male ratio of 1.5:1. Thirteen patients (11.1%) experienced regression. The incidence rate of spontaneous skin regression was 0.31 per 100 person-months and the average duration of SSc at the time of regression was 35.9 ± 15.6 months (range, 15.7-60 months). The factors that negatively correlated with regression were (a) diffuse cutaneous type, (b) Raynaud's phenomenon, (c) esophageal dysmotility, and (d) colchicine treatment at onset with a respective hazard ratio (HR) of 0.19, 0.19, 0.26, and 0.20. By contrast, the factor that positively correlated with regression was active alveolitis with cyclophosphamide therapy at onset with an HR of 4.23 (95% CI, 1.23-14.10). After regression analysis, only Raynaud's phenomenon at onset and diffuse cutaneous type had a significantly negative correlation to regression. A spontaneous regression of the skin fibrosis process was not uncommon among Thai SSc patients. The factors suggesting a poor predictor for cutaneous manifestation were Raynaud's phenomenon, diffuse cutaneous type while early cyclophosphamide therapy might be related to a better skin outcome. [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]

Systemic sclerosis (SSc) is recognized as a chronic inflammatory disease and several SSc-associated factors may increase the risk of osteoporosis and its related fractures. To determine the prevalence and predictive factors of osteoporosis in Thai SSc, a cross-sectional study was designed in adult SSc patients at Scleroderma clinic, Khon Kaen University Hospital. The prevalence of osteoporosis with the 95% confidence interval (CI) were determined and the odds ratio (OR) with 95%CI were assessed the clinical association with osteoporosis. A total of 205 SSc patients were recruited with the female to male ratio of 2.7:1. The majority of cases were diffuse SSc subset (83.4%) with a disease duration < 5 years (62.9%). The overall prevalence of osteoporosis was 29.3% (95%CI 23.1–36.0). After an age adjusted analysis, the respective prevalence of osteoporosis at lumbar spine (LS) in women and men was 26.3% and 10%, while the prevalence of osteoporosis at the femoral neck (FN) in women and men was 11% and 2.1%. Low BMI (≤ 18.5 kg/m2) and menopause were associated with osteoporosis at both the LS and FN. Using multivariate analysis, low BMI and menopause were associated with osteoporosis at LS (OR 7.78 and 5.32, respectively), while low BMI was also associated with osteoporosis at LS in pre-menopausal women. In conclusion, the prevalence of osteoporosis in Thai SSc was 29.3%. Osteoporosis at the LS is more common than FN in both men and women. Low BMI was associated with osteoporosis in overall SSc and pre-menopausal women, while only menopause was associated with osteoporosis at the FN. [ABSTRACT FROM AUTHOR]

Objectives: Pulmonary hypertension (PH) is a major cause of death in systemic sclerosis (SSc). Detection of early-onset PH and its associated factors would be helpful for improving patient care. Our aims were to determine the factors associated with early-onset PH and to define the differences between early- and late-onset PH among SSc patients. Methods: A cohort study was conducted of 409 adult SSc patients who had followed-up between January 2014 and December 2016. Early-PH is defined when the onset of PH is diagnosed within 5 years of the disease. Logistic regression analysis was applied to determine the factors associated with early-PH. Results: In 3409 person-years, we diagnosed 50 cases with PH confirmation by right heart catheterization, of whom 26 were early-PH (incidence 0.7 per 100 person-years; 95%CI:0.5-1.1). Among SSc with early-PH, 69.2% had the diffuse cutaneous SSc subset and the most common PH classification was PH due to interstitial lung disease (18 cases;69.2%). According to a logistic regression analysis, early-PH was associated with a WHO functional class (WHO-FC) II and higher, cardiomegaly according to chest radiography, and tricuspid regurgitation jet maximum velocity (TRVmax)>2.8 m/s with the respective OR of 20.12 (95%CI:1.59-255.35), 7.42 (95%CI:1.35-40.88), and 8.20 (95%CI:1.17-57.64). To contrast, early-PH had a negative association with gastrointestinal involvement (OR 0.08; 95%CI:0.01-0.56). Conclusions: Early-PH is prevalent among SSc patients and the most common cause is interstitial lung disease. A poor WHO-FC, cardiomegaly, and a high TRVmax are associated with early-PH. Gastrointestinal involvement is a protective factor for early-PH in SSc. [ABSTRACT FROM AUTHOR]

Background: Most Thai patients with systemic sclerosis (SSc) have diffuse cutaneous SSc (dcSSc) unlike most Caucasians and some Asians. A longitudinal cohort study among Thai dcSSc is needed. Objectives: We aimed to determine the overall clinical characteristics, define the clinical difference between limited cutaneous SSc (lcSSc) and dcSSc, and ascertain the mortality rate and the factors associated with mortality. Method: We conducted a cohort study including 566 Thai adult SSc patients between January 2013 and June 2019. Clinical difference between lcSSc and dcSSc was investigated using generalized estimating equations (GEE). Results: Females presented more than males (356 vs 210 cases). The majority of cases were dcSSc (411; 72.6%). The median duration of disease at the time of pulmonary fibrosis (PF) detection was 2.5 years, pulmonary arterial hypertension 8.1 years, and renal crisis 4.1 years. By GEE analysis, dcSSc was significantly associated with salt‐and‐pepper skin, hand deformity, and every 1‐point increase in modified Rodnan skin score (mRSS). A greater mortality risk was associated with age at onset >60 years (hazards ratio [HR] 5.5), a World Health Organization functional class (FC) III (HR 5.1), FC IV (HR 34.8), edematous skin (HR 11.4), early onset of PF (HR 1.7), each 5‐point increase in the mRSS (HR 4.5), and ≥2 internal organ involvements (HR 10.1). Conclusion: dcSSc is a common SSc subset among Thais. PF was an early complication in SSc and earlier PF detection was associated with a poorer prognosis. Elderly onset, high FC, severe skin tightness, and multiple organ involvements were associated with a greater mortality risk. [ABSTRACT FROM AUTHOR]

Background: Cardiac magnetic resonance imaging (cardiac MRI) has high sensitivity and specificity for differentiating cardiac fibrosis from inflammation. There is no data on the clinical and laboratory association or prediction of myocardial inflammation in systemic sclerosis—a major organ involvement in systemic sclerosis (SSc). Objectives: Our aim was to ascertain the clinical and laboratory associations with myocardial inflammation in SSc patients as detected by cardiac MRI. Methods: A cross‐sectional study was conducted among Thai adult SSc patients who had: disease onset <4 years; a New York Heart Association functional class ≥ II; and followed up at the Scleroderma Clinic, Khon Kaen University, between June 2018 and January 2019. We excluded patients who were taking steroids and/or immunosuppressants or had a diagnosed heart disease before being diagnosed with SSc. All enrolled patients underwent cardiac MRI, and clinical and laboratory assessments the same date. Myocardial inflammation was defined by cardiac MRI per the Lake Louise Criteria. Results: A total of 30 SSc patients were enrolled. The female‐to‐male ratio was 1.8:1. The majority (73%) had diffuse cutaneous SSc. The respective mean age and median duration of disease was 57 ± 8 and 2.0 years (interquartile range 1.5‐2.7). Myocardial inflammation was detected in 22 patients (73.3%). The multivariate analysis revealed that every 5 years of increased age at onset and every 5‐point increase in the modified Rodnan skin score (mRSS) at onset was significantly associated with myocardial inflammation (odds ratio 0.47, 95% CI 0.22‐0.98; and 2.64 95% CI 1.04‐6.74, respectively). Neither the SSc subset, internal organ involvement, inflammatory markers, nor cardiac and muscle enzymes were associated with myocardial inflammation in SSc. Conclusion: Myocardial inflammation is common in early‐onset SSc. An increased risk of myocardial inflammation was associated with young age and high mRSS at onset. Cardiac MRI is the suggested evaluation for high‐risk SSc patients experiencing dyspnea on exertion. [ABSTRACT FROM AUTHOR]

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]

Aims Skin tightness progression determines the severity and mortality of systemic sclerosis ( SSc). The modified Rodnan skin score ( mRSS) is a skin tightness assessment tool, albeit inter-physician variability is a limitation. Our objectives were to evaluate the correlation and agreement of skin tightness assessment between patient self-assessment and physician-assessment. Methods A descriptive study was conducted on the masked, self-assessments of mRSS (at week 0, 4 and 12) by 23 Thai adult SSc patients seen at Srinagarind Hospital, KhonKaen University, between March 2014 and February 2015. Correlation between the physician and patient assessments was estimated using Pearson's. The intra-class correlation coefficient ( ICC) and limit of agreement by Bland-Altman were determined. Results We included 23 SSc patients (female to male ratio being 1.1:1). All had the diffuse cutaneous SSc subset. The skin thickness assessment correlation was moderate at baseline (Pearson's r = 0.68) and improved to a good correlation at week 4 and 12 ( r = 0.78 and 0.86, respectively). The ICC showed good agreement (0.761) at week 0 and increased to excellent at week 4 (0.846) and 12 (0.910). The patient self-assessment score was higher than the physician assessment, which slightly decreased over time: mean difference = 4.30 (95% LOA;−9.54 to 18.14) at week 0, then 3.78 (95% LOA,−10.20 to 17.77) at week 4, and 3.16 (95% LOA,−7.78 to 14.10) at week 12. Conclusion The respective modified Rodnan skin score assessment by patient versus the physician was highly correlated with a high level of agreement. Validation of generalized patient self-assessment needs to be confirmed. [ABSTRACT FROM AUTHOR]

Background Scleroderma with characteristics of other connective tissue diseases is called scleroderma overlap syndrome ( SOV); the clinical features of which have yet to be investigated among Thai patients. Objective To determine the clinical differences between pure scleroderma and SOV. Methods A historical cohort study was conducted among patients with pure scleroderma versus those with SOV. Subjects were over 18 years of age and followed up at Srinagarind Hospital, Khon Kaen University, Thailand, between January 2006 and December 2011. Results Four hundred and three medical records were included (276 female vs. 127 male). SOV was found in 68 cases (16.9%): (i) scleroderma-polymyositis overlap ( SOV- PM), the most common type of SOV (48 cases; 70.6%); (ii) scleroderma-systemic lupus erythematosus overlap (11 cases; 16.2%); and (iii) scleroderma-rheumatoid arthritis overlap (nine cases; 13.2%). Mean age at onset of non-systemic sclerosis symptoms was 46.9 ± 11.8 years (range, 19.8-74.3). Characteristically, sufferers of SOV as against pure scleroderma were younger, had less frequent anti-topoisomerase I ( ATA) and needed moderate- to high-dose steroid and immunosuppressant therapy during follow-up. SOV- PM presented the clinical features of scleroderma at onset and during follow-up looks like pure scleroderma having vasculopathy, severity of skin tightness, and gastrointestinal, cardiopulmonary and renal involvement. Anti-Ro52 was the most common serology among sufferers of SOV (31.6%). ATA was associated with pure scleroderma patients ( P = 0.047). Conclusions SOV rather than pure scleroderma presented in younger Thai scleroderma patients and SOV- PM was the most common subtype and its clinical features were similar to those of pure scleroderma. ATA was strongly associated with the latter. [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]

Purpose: To evaluate the prevalence of dry eye disease (DED) in patients with both limited and diffuse subtypes of systemic sclerosis (SSc) and to determine the predictive factors associated with the occurrence of DED. Patients and Methods: This was a prospective consecutive cross-sectional study of patients with SSc. Each patient underwent a comprehensive ophthalmological evaluation. The Ocular Surface Disease Index (OSDI) was used to assess the symptoms of DED. Tear break up time (TBUT), Schirmer I and ocular surface staining (OSS) were used for objective tests. Patients were diagnosed DED using the DEW II diagnostic method which included both symptom and objective tests. The collected data of patients with and without DED were compared to find out possible associated factors. Logistic regression analysis was used to determine the effects of these factors. Results: Eighty-four SSc patients (25 limited and 59 diffuse subtypes) were studied. The prevalence of DED in SSc patients was 52.38%. The OSDI, tear break up time (TBUT), Schirmer I and ocular surface staining (OSS) were not significantly different between SSc subtypes. Among the 46 symptomatic patients, DED was noted in 44 (52.38%). Most patients (61.4%) were mixed aqueous deficiency-evaporative dry eye type (both TBUT and Schirmer I test positive). The mean age and the median of disease duration in DED patients were higher than those without DED with statistically significant difference (P = 0.004 and 0.019). Conclusion: DED was common in patients with SSc. The predictors for the occurrence of DED were older age and longer disease duration. Therefore, OSDI and objective tests for DED should be evaluated in all SSc patients, particularly those with older age and longer disease duration. [ABSTRACT FROM AUTHOR]

Introduction: Anti-neutrophilic cytoplasmic antibody (ANCA) has been reported in systemic sclerosis (SSc). Some clinical features of SSc can also be presented with ANCA-associated vasculitis. Objectives were to determine the prevalence and clinical associations with ANCA among Thais with SSc.Material and Methods: A cross-sectional study of Thai adult SSc patients was conducted. According to the sample size calculation, 185 patients were included. Clinical and laboratory tests for serology and others for evaluation of the clinical association with ANCA were done simultaneously on the study date.Results: The female to male ratio was 2 : 1. The majority had the diffuse SSc subset (71.2%). The respective prevalence of having a) at least 1 serological test for ANCA (viz., perinuclear ANCA, cytoplasmic ANCA, anti-myeloperoxidase, or anti-proteinase3), b) positive for either p-ANCA or c-ANCA, c) positive for either anti-MPO or anti-PR3, d) p-ANCA and anti-MPO and e) c-ANCA and anti-PR3 was 21.6% (95% CI: 15.9-28.3), 11.9% (95% CI: 7.6-17.4) and 13.0% (95% CI: 8.5-18.7) and 1% (95% CI: 0.1-3.9). By multivariate analysis, the erythrocyte sedimentation rate elevation was significantly associated with the presence of the antibody (OR = 11.36, 95% CI: 1.44-83.65), while elevation of high sensitivity cardiac troponin-T (hs-cTnT) was significantly associated with the presence of either p-ANCA or c-ANCA (OR = 4.25, 95% CI: 1.41-15.34). None of the patients had clinical features of systemic vasculitis.Conclusions: Around one-fifth of SSc patients have detectible ANCA without any features of vasculitis. The presence of ANCA is associated with inflammation and myocardial injury. ANCA is not antibody specific for vasculitis in SSc. [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]

Thyroid disease, particularly hypothyroidism, has been reported in systemic sclerosis (SSc). Some clinical features of SSc can also present in hypothyroidism. Our aims were to determine the prevalence of, and describe clinical features associated with, hypothyroidism in SSc patients. We conducted a historical cohort study of adult SSc patients who underwent screening thyroid function tests at the Scleroderma Clinic, Khon Kaen University, Thailand, between 2009 and 2018. The patients who had any thyroid disorders before the onset of SSc and were diagnosed as an overlap syndrome were excluded. A total of 200 SSc were included according to sample size calculation, among whom the female to male ratio was 2:1. The majority of cases (137; 69.5%) were diffuse cutaneous SSc subset. The mean age was 55.8 ± 10.7 years and the median duration of disease 4.9 (IQR 1.6–9.9) years. Of the total, 9 had primary hypothyroidism (prevalence 4.5%; 95%CI 2.1–8.4) and 22 had subclinical hypothyroidism (prevalence 11%; 95%CI 7.0–16.2). Of the latter 22, 71% had dcSSc. Logistic regression analysis indicated that unexplained anemia was significantly associated with either subclinical hypothyroid or hypothyroidism (OR 2.74; 95% CI 1.17–6.47), whereas Raynaud's phenomenon had a negative association (OR 0.28; 95% CI 0.11–0.66). Neither severity of skin tightness nor internal organ involvement were associated with hypothyroidism among SSc patients. Clinical-subclinical hypothyroidism is uncommon among SSc patients, it is frequently associated with anemia, and less so Raynaud's phenomenon. Clinical-subclinical hypothyroidism should thus be considered in cases of unexplained anemia in SSc patients. [ABSTRACT FROM AUTHOR]

The low prevalence of pregnancy in women with systemic sclerosis (SSc) is due to multi-factorial causes, including premature ovarian insufficiency (POI). The study aimed to determine the prevalence of POI, early menopausal status, and any clinical associations of these among Thai female SSc patients. An analytical cross-sectional study was conducted among female SSc patients between 18 and 45 years of age. The eligible patients underwent blood testing for follicle stimulating hormone and anti-mullerian hormone levels, gynecologic examination, and transvaginal ultrasound for antral follicle count. We excluded patients having surgical amenorrhea, previous radiation, and history of hormonal contraception < 12 weeks and pregnancy. A total of 31 patients were included. The majority (67.7%) had diffuse cutaneous systemic sclerosis. Three patients were POI with a prevalence of 9.7%. The factors associated with POI were a high cumulative dose of cyclophosphamide (CYC) (p = 0.02) and the long duration of CYC used (p = 0.02). After excluding POI, early menopause was detected in 10 patients with a prevalence of 35.7%. The factors associated with early menopause were long disease duration (p = 0.02), high cumulative dose of CYC (p = 0.03), and high cumulative dose of prednisolone (p = 0.02). Low ovarian reserve according to POSEIDON definition was found in 28 patients with the prevalence of 90.3%. POI in Thai SSc was uncommon, whereas early menopause and low ovarian reserve were frequently revealed. A high cumulative dose of CYC was associated with both POI and early menopause. Physicians should be aware of reproductive outcomes and advise patients at risk. Key Points •POI is revealed in patients with SSc particularly in who received high cumulative dose of cyclophosphamide, while early menopause and low ovarian reserve were major reproductive problem among SSc. • Prescriptions for CYC for female SSc—both for young patients of reproductive age and premenopausal middle-aged women—should be concerned of the long-term effects on gonadal function. [ABSTRACT FROM AUTHOR]

Occupational and environmental associations with systemic sclerosis (SSc) have been confirmed; however, the association between aerosol components and mortality is uncertain. The study aimed to define the association between aerosol components and hospital mortality among Thai SSc patients. A study was conducted using a national database of patients covered by the National Health Security Office, hospitalised between 2014 and 2018. Data included all patients over 18 having a primary diagnosis of SSc (ICD-10: M34). Spatial resources used map information based on GPS coordinates of Thailand. Aerosol components—including organic carbon, black carbon, dust particulate matter diameter < 2.5 µm (PM2.5), and sulfate—were assessed using the NASA satellite MERRA-2 Model M2TMNXFLX v5.12.4. Spatial modelling with R Package Integrated Nested Laplace Approximation (R-INLA) was used to analyse the association between the incidence of mortality and the 5-year accumulation of each aerosol component adjusted by age, sex, and comorbid diseases. The study included 2,094 SSc patients with 3,684 admissions. Most (63.8%) were female. During admission, 1,276 cases died. R-INLA analysis indicated an increase of 1 µg/m3 of dust PM2.5 was associated with a respective increase in the risk of overall mortality and death due to pneumonia of 96% and 79%. An increase of 1 µg/m3 of dust PM2.5 resulted in 1.17, 1.18, 1.64, and 2.15 times greater risk of mortality due to pulmonary fibrosis, cardiac involvement, renal involvement, and cancer, respectively. Aerosol components—particularly dust PM2.5 exposures—increased the risk of overall, cardio-pulmonary-renal, and cancer mortality among SSc patients. [ABSTRACT FROM AUTHOR]

Background. Myocarditis is reported in systemic sclerosis (SSc); however, treatment options and outcomes are limited. Our objective was to define cardiac outcomes after moderate-dose steroid therapy in SSc patients with myocarditis. Method. An open-label study was conducted among SSc patients with myocarditis—as defined by cardiovascular magnetic resonance (CMR), disease onset <5 years, and a NYHA functional class ≥II. All enrolled patients received prednisolone (30 mg/d) which would be tapered off by week 24, and CMR was followed up at the end of treatment. Results. A total of 20 SSc patients were enrolled which 12 patients completed the study. At week 24, 8 of the 12 cases experienced improvement of myocarditis. Compared to those with no improvement, these 8 patients had significantly longer disease duration (p = 0.03), higher heart rate at baseline (p = 0.049) and week 24 (p = 0.04), lower left ventricular (LV) and right ventricular (RV) stroke volume at baseline (p = 0.002 and p = 0.01) and week 24 (p = 0.01 and p = 0.02), and lower LV and RV cardiac output at week 24 (p = 0.01 and p = 0.01). Four cases died during follow-up (3 due to cardiac complications, 1 due to renal crisis). The two who died from heart failure had very high NT-prohormone-brain natriuretic peptide (NT-proBNP) and impaired LV ejection fraction (LVEF), and the one who died from arrhythmia had very high sensitivity of cardiac Troponin-T (hs-cTnT). Conclusions. Moderate-dose steroid therapy may improve myocarditis in SSc. A proportion of patients died due to cardiac complications during treatment, particularly those with high hs-cTnT, high NT-proBNP, and impaired LVEF. This trial is registered with NCT03607071. [ABSTRACT FROM AUTHOR]

Rapidly progressive interstitial lung disease (RP-ILD) and its distinctive cutaneous features are highly associated with the presence of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody in patients with dermatomyositis (DM), leading to a poor prognosis. We describe the case of a 25-year-old man who developed progressive proximal muscle weakness with RP-ILD and had unusual cutaneous findings (cutaneous ulcerations and livedo reticularis) accompanied by classical cutaneous features (heliotrope rash, Gottron's papules, Gottron's sign, and flagellate erythema). Blood test was positive for anti-MDA5 antibody. He was treated with intravenous corticosteroids and immunoglobulin, but passed away due to respiratory failure within 1 month after admission. Our case highlights that the presence of cutaneous ulcerations and livedo reticularis, in addition to RP-ILD, are useful clinical clues that may aid in the detection of anti-MDA5 antibody, early initiation of combined immunosuppressants, and prognosis prediction in patients with classical DM. [ABSTRACT FROM AUTHOR]

Abstract: Aim: To evaluate and compare the retention rate of biological disease‐modifying antirheumatic drugs (bDMARDs) in real‐life practice and identify risk factors related to remission and drug discontinuation in patients with rheumatoid arthritis (RA). Method: A total of 256 patients fulfilling criteria for RA and starting bDMARD between December 2009 and October 2014 were selected from the Rheumatic Disease Prior Authorization registry. Baseline demographic and clinical data were recorded. The cumulative probability of bDMARD discontinuation over 5 years of follow‐up and factors associated with RA remission and bDMARD withdrawal were analyzed. Results: Almost half (46%) of patients were initially treated with rituximab (RTX), with 33% treated with etanercept (ETN) and 21% with infliximab (IFX). Fewer than 10% were subsequently switched to a second bDMARD. The 1‐ and 5‐year remission rates in patients continuing their first bDMARD were 7.2% and 21.5%, respectively. At 5 years, the drug survival rates for RTX, ETN and IFX were 50%, 25% and 22%, respectively. Multivariate analysis showed that RTX was significantly associated with highest drug survival. Relative to RTX, the hazard ratios for discontinuation of IFX and ETN were 2.60 (95% confidence interval [CI] 1.53–4.42) and 2.15 (95% CI 1.36–3.42), respectively. Thirty‐nine percent of patients stopped treatments, due to inadequate response (42%), serious adverse events (22%), nonadherence (14%) or remission/low disease activity (13%). Conclusion: Over 5 years, only one‐third of patients continued using their first bDMARD. The leading cause of drug discontinuation was inadequate response. [ABSTRACT FROM AUTHOR]

Objective Autoantibody profiles in systemic sclerosis ( SSc) and their relative clinical association vary between studies. The rate for being anti-topoisomerase-I ( ATA) positive and the association with diffuse cutaneous the SSc subset (dc SSc) is higher among Thais than among Caucasians. The objective was to evaluate the relevance of clinical presentation, namely being positive for one or more autoantibodies among Thai SSc patients. Method A retrospective, cohort study was performed among SSc patients over 18 years of age at Srinagarind Hospital, Khon Kaen University, Thailand, during January 2006 to December 2013. Autoantibodies comprising 13 SSc-specific antigens were evaluated using the EUROIMMUN AG (Lübeck, Germany) in order to define their clinical association(s). Results Two hundred and eighty-five scleroderma patients (200 female; 85 male) were included. The majority (66.7%) were dc SSc subset. ATA was the most common antibody profile in our patients (231 cases; 81.1%), followed by anti-Ro 52 (87 cases; 30.5%). Eleven of our patients (3.9%) were negative for all antibody profiles and 44 cases (15.4%) were negative for ATA and anti-centromere antibody (anti- CENP). Almost 40% (112 cases) were positive for at least two autoantibodies. There was an association between the presence of ATA and hand deformity (odds ratio [ OR] 3.94; 95% CI 1.12-13.84), anti- CENP and hand deformity ( OR 0.20; 95% CI 0.02-0.90), anti-Ku and scleroderma-polymyositis overlap syndrome ( OR 6.58; 95% CI 2.16-19.39) and the absence of both ATA and anti- CENP with female sex ( OR 2.90; 95% CI 1.12-7.51), limited cutaneous SSc subset ( OR 2.70; 95% CI 1.30-5.55) and scleroderma-polymyositis overlap syndrome ( OR 2.53; 95% CI 1.04-6.16). Neither ATA nor anti- CENP were associated with the SSc subset. Conclusions ATA and anti- CENP were not helpful in differentiating the SSc subset in Thai SSc patients, albeit they were good for predicting hand function. Coexisting ATA and anti- CENP negativity were associated with less extensive skin tightness and SSc overlap syndrome. [ABSTRACT FROM AUTHOR]

Aim In June 2015, the Thai Rheumatism Association (TRA) approved an update of its recommendation for the use of biologic disease-modifying anti-rheumatic drugs (bDMARDs) and targeted synthetic (tsDMARD) in the treatment of rheumatoid arthritis (RA) to cover those currently available in Thailand (etanercept, infliximab, golimumab, rituximab, tocilizumab, abatacept and tofacitinib). Method A search of the literature was performed between January 2000 and June 2015. Existing RA recommendations, in relation to the use of bDMARDs and tsDMARD, were identified and evaluated by the AGREE II instrument prior to their use as a 'guide' for developing this TRA recommendation. An additional literature search was performed in order to answer specific clinical questions that could not be found in existing guidelines. Result Thirteen recommendations were developed. They covered the use of RA classification criteria, the aim of RA treatment, when to initiate bDMARDs/tsDMARD or taper or switch them to other medications, as well as monitoring these drugs during their use. In addition, specific issues including their use and vaccination, malignancies, pregnancy and lactation, and perioperative period also were addressed. Public hearings were performed at the annual meeting of the TRA and of the Royal College of Physicians of Thailand. The recommendations were distributed to other professional associations related to RA management, as well as government sectors associated with the reimbursement policy, prior to development of the final version. Conclusion These recommendations will help Thai rheumatologists prescribe bDMARDs and tsDMARD more appropriately when treating RA patients. [ABSTRACT FROM AUTHOR]

Aim Rheumatoid arthritis ( RA) is a chronic inflammatory joint disease leading to joint damage, functional disability, poor quality of life and shortened life expectancy. Early diagnosis and aggressive treatment are a principal strategy to improve outcomes. To provide best practices in the diagnosis and management of patients with RA, the Thai Rheumatism Association ( TRA) developed scientifically sound and clinically relevant evidence-based recommendations for general practitioners, internists, orthopedists, and physiatrists. Methods Thirty-seven rheumatologists from across Thailand formulated 18 clinically relevant questions: three for diagnosis, 10 for treatments, four for monitoring, and one for referral. A bibliographic team systematically reviewed the relevant literature on these topics up to December 2013. A set of recommendations was proposed based on the results of systematic reviews combined with expert opinions. Group consensus was achieved for all statements and recommendations using the nominal group technique. Results A set of recommendations was proposed. For diagnosis, either American College of Rheumatology ( ACR) 1987 or ACR/European League Against Rheumatism 2010 classification criteria can be applied. For treatment, nonsteroidal anti-inflammatory drugs, glucocorticoid, and disease-modifying antirheumatic drugs, including antimalarials, methotrexate and sulfasalazine are recommended. Physiotherapy should be suggested to all patients. Tight control strategy and monitoring for efficacy and side effects of treatments, as well as indications for referral to a rheumatologist are provided. Conclusions These evidence-based recommendations provide practical guidance for diagnosis, fundamental management and referral of patients with RA for non-rheumatologists. However, it should be incorporated with clinical judgments and decisions about care for each individual patient. [ABSTRACT FROM AUTHOR]

Background Anti-topoisomerase I antibody ( ATA) carries an increased risk of systemic sclerosis ( SSc) internal organ involvement. There have been no published comparisons of the clinical characteristics of patients positive and negative for ATA in Thailand, where the positive rate for ATA is higher than among Caucasians. Objective To define the clinical differences between SSc, positive versus negative, for ATA. Methods A retrospective cohort study was performed among SSc patients over 18 at Srinagarind Hospital, Khon Kaen University, Thailand, during January 2006-December 2013. SSc-overlap syndrome was excluded. Results Two hundred and ninety-four SSc patients were included (female : male 2.5 : 1). The majority (68.6%) were the diffuse cutaneous SSc subset (dc SSc). ATA was positive in 252 patients (85.7%), among whom 71.7% had dc SSc and 28.2% limited cutaneous SSc (lc SSc). Using a multivariate analysis, hand deformity had a significantly positive association with ATA (odds ratio [ OR] 7.01; 95% CI 1.02-48.69), whereas being anti-centromere ( ACA) positive had a negative association ( OR 0.17; 95% CI 0.03-0.92). After doing a subgroup analysis of the SSc subset, the median duration of disease at time of pulmonary fibrosis detection among ATA positive dc SSc was significantly shorter than the ATA negative group (1.05 vs. 6.77 years, P = 0.01). Raynaud's phenomenon ( RP) at onset was significantly more frequent in lc SSc sufferers who were ATA negative than those who were ATA positive (90.5% vs. 56.9%, P = 0.005). Conclusions A high prevalence of ATA positivity was found among Thai SSc patients and this was associated with a high frequency of hand deformity, ACA negativity, a short duration of pulmonary fibrosis in dc SSc and a lower frequency of RP in lc SSc. [ABSTRACT FROM AUTHOR]

Introduction: Spirometry is a screening tool for evaluating the degree of restrictive lung disease in systemic sclerosis (SSc). Observations indicated that some patients could not complete the test. The aim of the study was to identify the prevalence, causes and clinical predictors of an inadequate pulmonary function test (PFT) in SSc.Material and Methods: A cross-sectional study was performed among SSc patients over 18 years old followed up at Srinagarind Hospital, Khon Kaen, Thailand, during January 2006-December 2012. The adequacy of the PFT was based on the acceptable blow criteria as set out by the American Thoracic Society and the European Respiratory Society 2005 Standardizations of Spirometry.Results: Two hundred and forty-nine patients were included (female to male ratio was 2 : 1). The mean age at performing PFT was 51.4 ±11.1 years (range: 19.6-79.5). Median duration of disease at performing PFT was 2 years (IQR: 0.6-4.4). Inadequate PFT occurred in 73 cases (prevalence 29.3%: 95% CI: 23.6-35.0); the majority (60 cases; 82.2%) had an expiration time < 6 s and the others were due to plateau < 1 s (11 cases; 15%), air leak around mouth piece (1 case; 1.4%) and hesitation (1 case; 1.4%). Thirteen of 73 (17.8%) had an unusable graph with the overall prevalence of 5.2% (95% CI: 2.4-8.0). The factor associated with inadequate PFT was docy mass index (BMI) < 18.5 kg/m(2) (OR = 2.17: 95% CI: 1.49-3.17); the same factor was associated with an unusable graph, which was confirmed by the multivariate analysis (OR = 5.21; 95% CI: 1.60-16.95).Conclusions: One-third of Thai SSc patients had an inadequate pulmonary function test - the majority because of inadequate time for expiring. Low BMI influenced the effectiveness of the test, leading to an incomplete graph for evaluating lung disease in SSc. [ABSTRACT FROM AUTHOR]

Background: The major cause of death in systemic lupus erythematosus (SLE) is due to the disease activity itself or infection. It is uncertain whether the treatment approach during the last decade prolonged survival in SLE. Objective: Our objective was to identify the causes of death and the factors predictive of mortality and to calculate the survival rate among SLE patients. Method: We conducted a retrospective study of SLE patients followed up at Srinagarind Hospital, Khon Kaen University, Thailand, between January 1, 1996 and August 31, 2005. Cox regression analysis was used to estimate the probability of survival and assessing factors associated with death. The medical records of 749 SLE patients were reviewed; 66 patients died during the follow-up period. Results: The mortality rate was 1.2 per 100 person-years. The 5- and 10-year survival rate among our SLE patients was 93% and 87%, respectively. The mean age at death was 34.08 ± 11.75 years and the median disease duration was 48 (1-336) months. One-third of the cases were referred from a local hospital more than 1 month after onset and were associated with a significantly higher risk of mortality than cases referred earlier ( P = 0.047). The most common causes of death were opportunistic pulmonary infections and neuropsychiatric lupus. Factors predictive of mortality included: (i) major organ flare more than four times per year; (ii) age at onset > 50 years; (iii) high-dose steroid use and/or immunosuppressive therapy at onset and within 2 weeks prior to death; and (iv) concomitant diabetes mellitus. Protective against mortality was antimalarial use. [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]

Background: Undifferentiated arthritis (UA) comprises arthritis not yet identifiable as a specific rheumatic disease. Few reports exist on the natural course of UA in Thai patients. Objective: To study the clinical features and natural course of UA in Thai patients. Method: A retrospective, analytical study was performed among Thai patients diagnosed with UA seen at Srinagarind Hospital, Khon Kaen, Thailand, between January 2002 and December 2007. Results: The medical records of 95 UA patients were reviewed. The mean age at onset was 40.7 ± 14.7 years (range, 15-78). The female:male ratio was 1.25 : 1.00. Common presentations included asymmetrical oligoarthritis followed by polyarthritis. The knee was the most commonly affected joint, followed by the wrist and ankle. Complete remission occurred within 6 months of onset in 4.2% of cases. A diagnosis was specified in 29 patients (30.5%) during the follow-up period (which averaged 17.1 ± 24.0 months [range, 6-84]), including reactive arthritis (in 9 patients), undifferentiated spondyloarthropathy (7), rheumatoid arthritis (6), psoriatic arthritis (4), ankylosing spondylitis (1), gout (1) and unclassified connective tissue disease (1). UA was the default diagnosis for 66 patients (69.5%) after 24 months of follow-up. Hyperglobulinemia was correlated with persistent arthritis (i.e., > 6 months, P = 0.045). The only predictive factor for RA development was old-age at onset ( P = 0.038). Conclusion: The most common presentation of Thai UA was asymmetrical oligoarthritis and most patients had persistent arthritis correlated with hyperglobulinemia. Elderly-onset, without any radiographic changes or rheumatoid factor, was predictive of RA development during follow-up. [ABSTRACT FROM AUTHOR]

Abstract: After the first dose injection of pegylated interferon alpha-2b (Peg-IFN alpha-2b) to a HCV infected Thai woman, she developed cyclic painful swelling nodules on right upper quadrant of abdomen and right anterior lower chest wall. The nodules subsided spontaneously within 1–2 days but were recurrent after every Peg-IFN alpha-2b injection. She also experienced acute urticaria. After nine months of therapy, an immature male of G. spinigerum migrated out from the skin nodule shortly after a Peg-IFN alpha-2b injection as scheduled. The worm showed a head-bulb bearing 8 transverse rows of spines which indicated immature stage. It had well defined four pairs of caudal papillae on posterior body part which were used to identify male gender. Painful migratory swelling and urticaria disappeared after the parasite was removed. She was continually treated and had sustained both virological and biochemical responses to HCV treatment. This case demonstrates that the outward migration of G. spinigerum may be stimulated by the injection of Peg-IFN alpha-2b. [Copyright &y& Elsevier]