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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]

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: 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]

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]

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]

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]

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]

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]