Levels of anti-topoisomerase I antibody correlated with short onset of cardiopulmonary involvement in Thai systemic sclerosis patients.

Anti-topoisomerase-I antibody (ATA) is associated with disease severity and internal organ involvement in patients with systemic sclerosis (SSc). The correlation between ATA levels and the clinical course of SSc is unclear. We aimed to determine the correlation between ATA level and survival time and the onset of internal organ fibrosis in SSc patients. This historical cohort study was conducted in adult SSc patients with quantitative tests of ATA between January 2019 and December 2022. Patients with overlap syndrome and no quantitative ATA test were excluded. According to the sample size calculation, and 10% compensated for missing data, a total of 153 patients were needed. The respective mean age on the study date and median ATA level was 59.9 ± 11.3 years and 370 U/mL (range 195-652). Most cases (107 cases; 69.9%) were the diffuse cutaneous SSc subset. According to a multivariable analysis, the ATA titer had a negative correlation with the onset of cardiac involvement (Rho - 0.47, p = 0.01), and had a positive correlation with skin thickness progression (Rho 0.39, p = 0.04). Eleven cases exhibited ATA levels < 7 U/mL and outlier ATA levels were excluded, 142 cases were included in the sensitivity analysis, and multivariable analysis showed the correlation between early onset of ILD and cardiac involvement (Rho - 0.43, p = 0.03 and Rho - 0.51, p = 0.01, respectively). The ATA level was correlated with neither the survival time nor the onset of renal crisis in both analyses. High ATA levels were correlated with a short onset of ILD and cardiac involvement and the presence of extensive skin tightness. Quantitative tests of ATA could serve as an effective tool for identifying patients at risk of an unfavorable prognosis.

Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody.

Purpose: Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) overlap with systemic sclerosis (SSc) is uncommon. We aimed to determine the incidence of AAV and define clinical outcomes relevant to asymptomatic screening positive for ANCA in SSc after 2 years of follow-up. Patients and Methods: The study was a cohort study of 185 Thai adult SSc patients testing for ANCA and having a 2-year follow-up at the Scleroderma Clinic, Khon Kaen University, Thailand. The incidence of AAV and outcomes of those who tested positive for ANCA were evaluated. Results: A total of 185 SSc patients were tested for ANCA, of whom 21.6% were positive for either cytoplasmic ANCA, perinuclear ANCA (p-ANCA), anti-myeloperoxidase (anti-MPO), or anti-proteinase3 antibody. Only one 52-year-old female patient with dcSSc, negative for initial ANCA test, developed AAV (microscopic polyangiitis) 7 months after the first ANCA test for an incidence of AAV of 0.27 per 100-person-years (95% CI 0.01-1.5). She was positive for p-ANCA and anti-MPO. Eight of those who had an initial test were positive for ANCA and underwent a repeated test. Only two cases persisted as positive for ANCA (1 anti-MPO and 1 anti-PR3) and had no clinicals suspicious of vasculitis. Four cases that had ANCA turned to a negative result. Conclusion: AAV is a rare complication in SSc, so ANCA may not have any role as a screening test for AAV as it cannot predict the development of AAV in SSc. We suggest testing for ANCA only in SSc patients with clinicals suspicious of AAV.

Incidence and Prevalence of Septic Arthritis in Thailand: A Database from the Ministry of Public Health.

Background: A better understanding of the epidemiological profile of septic arthritis or pyogenic arthritis in Thais could improve care and provide information for better infectious control. We aimed to determine the incidence and prevalence of septic arthritis in Thailand between 2017 and 2020. Methods: A descriptive epidemiological study was performed using demographic data from patients over 18 years of age having a primary diagnosis of M00 pyogenic arthritis between 2017 and 2020. Data were sourced from the Information and Communication Technology Center, Ministry of Public Health database. The incidence and prevalence of septic arthritis were calculated, and their respective 95% confidence interval (CI). Results: The number of patients with septic arthritis in 2017 was 26,878 from a total Thai population of 65,204,797. The prevalence of septic arthritis in 2017 was 41.2 per 100,000 (95% CI 40.7-41.7). The prevalence of septic arthritis among women was slightly higher than among men (42.2 vs 40.2 per 100,000). The incidence of septic arthritis slightly increased from 2018 to 2019 but was stable in 2020 (22.6, 23.3, and 23.1 per 100,000 person-years, respectively). The incidence was highest in the southern region between 2018 and 2019 but highest in the northeast in 2020. The peak was in the elderly population 60 and older (56.4, 59.5, and 57.3 per 100,000 person-years in 2018, 2019, and 2020, respectively). The incidence increased with age and the maximum rate was in those ≥ 70 years (70.2 per 100,000 person-years in 2019). Conclusion: Septic arthritis commonly presents in the elderly and is comparable between men and women. The disease was found mainly in the northeastern and southern regions. The incidence remained stable during the study period.

Cost-effectiveness of alginic acid in combination with proton pump inhibitor for the treatment of gastroesophageal reflux disease in systemic sclerosis patients.

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.

Sleep quality and clinical association with sleep disturbance in systemic sclerosis.

BACKGROUND: Poor sleep quality is a common and potentially debilitating problem in systemic sclerosis (SSc). To date, no data clarifies the potential factors related to poor sleep quality and the clinical associations with sleep disturbance among Thais with SSc-mainly the diffuse cutaneous SSc (dcSSc) subset. We aimed to evaluate sleep quality and identify the clinical association with sleep disturbance among SSc patients. METHODS: A cross-sectional study was conducted between May 2021 and September 2021. Adult SSc patients were enrolled at the Scleroderma Clinic, Khon Kaen University, Thailand. All patients had their neck circumference measured, underwent airway evaluation using the Mallampati classification, had sleep quality assessed using the Pittsburgh Sleep Quality Index (PSQI), and the Berlin and Patient Health Questionnaire-9 completed. In addition, the clinical association with poor sleep quality (or sleep disturbance) was investigated using the PSQI. RESULTS: A total of 88 patients were enrolled. Forty-eight (54.6%) patients experienced poor sleep quality (95%CI 43.6-65.2). Digital ulcers and dyspepsia were associated with poor sleep quality as per a logistic regression (OR 10.73: 95%CI 1.09-106.15 and 4.60: 95%CI 1.01-20.89), respectively. Overall pain-evaluated using the visual analog scale (VAS)-was positively correlated with the PSQI score (Rho 0.2586; p = 0.02). CONCLUSION: Around half of the SSc patients reported poor sleep quality, and the significantly associated factors were digital ulcers and dyspepsia. The PSQI scores positively correlated with overall pain as evaluated by VAS. With early assessment and treatment of digital ulcers, stomach symptoms, and pain control, sleep problems might be reduced among SSc patients.

Attitudes and factors affecting decision-making regarding COVID-19 vaccination among autoimmune rheumatic disease patients.

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.

Incidence and prevalence of systemic sclerosis in Thailand in year 2017-2020: a database from the Ministry of Public Health.

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.

Management review of scleroderma renal crisis: An update with practical pointers.

Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement therapy and mortality persists despite various treatments. Depending on the stage of SRC, a vasodilator called angiotensin-converting enzyme inhibitor is the treatment of choice. The efficacy of various other vasodilators (i.e. endothelin-1 receptor antagonist) and complement cascade blocker for SRC have been investigated; however, no randomized control trial has been conducted. A new approach has been proposed for the management of SRC, categorized by specific clinical features of narrowly defined SRC and systemic sclerosis-thrombotic microangiopathy. SRC prophylaxis using angiotensin-converting enzyme inhibitor might be harmful, leading to a poor renal outcome, so the pathogenesis of SRC needs to be clarified in order to identify other possible preventions or therapies.

Fingerprint comparison between before disease onset and after systemic sclerosis diagnosis: a monocentric cross-sectional study.

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.

Development and validation of machine learning for early mortality in systemic sclerosis.

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.

Prevalence and clinical associations with primary hypogonadism in male systemic sclerosis.

Background: Systemic sclerosis may affect male and female fertility. Premature ovarian failure has been reported in female systemic sclerosis patients, but the effects on male fertility in systemic sclerosis have not been studied. Objectives: We aimed to determine the prevalence and clinical associations with primary hypogonadism among male systemic sclerosis patients. Methods: This was a cross-sectional pilot study, including 30 adult male systemic sclerosis patients attending the Scleroderma Clinic, Khon Kaen University. Testosterone deficiency symptoms were assessed using the Aging Males' Symptoms Rating Scale, urological examination, and blood testing (for total testosterone, free testosterone, follicle-stimulating hormone, and luteinizing hormone). We excluded patients with congenital hypogonadism and any acquired disorders of the testes and genitalia. The definition of primary hypogonadism was based on the International Society for the Study of the Aging Male 2015 diagnostic criteria for hypogonadism. Results: Seven patients met the definition of primary hypogonadism-a prevalence of 23.3% (95% confidence interval: 9.9-42.3). The respective mean age and mean systemic sclerosis duration was 59.4 ± 11.9 and 5.5 ± 4.7 years. Older age at onset, high triglyceride level, and older age starting corticosteroid treatment were significantly associated with primary hypogonadism (p = 0.02, 0.02, and 0.03, respectively). Systemic sclerosis subset, disease severity, and immunosuppressant use were not associated with primary hypogonadism among Thai male systemic sclerosis patients. Conclusion: Around one-quarter of male systemic sclerosis patients had primary hypogonadism. Elderly onset of systemic sclerosis, hypertriglyceridemia, and late corticosteroid treatment were risk factors for developing primary hypogonadism.

Predictors for Dry Eye Diseases in Patients with Systemic Sclerosis.

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.

Prevalence and clinical association of sarcopenia among Thai patients with systemic sclerosis.

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.

Skin thickness affects the result of tuberculin skin test in systemic sclerosis.

BACKGROUND: Skin thickness is a prominent clinical feature of systemic sclerosis (SSc), but there is no consensus on the cut-off for a positive tuberculin skin test (TST) size and the limitation of the TST for a diagnosis of tuberculosis in SSc. We aimed to identify the cut-off size of an indurated TST and the sensitivity and specificity of the test for the diagnosis of tuberculosis in SSc patients. METHODS: A cross-sectional study of 168 adult Thai SSc patients was conducted. The TST was done using 0.1 ml of purified protein derivatives via intradermal injection. The test was interpreted 72 h after testing. RESULTS: The median age was 57.2 years. The majority (71.8%) had the diffuse cutaneous SSc subset. All the patients had a BCG vaccination at birth, and 17 (10.1%) had a tuberculosis infection. An indurated skin reaction size of 20 mm had the highest specificity for tuberculosis (99.3%: 95%CI 96.4-100) (ROC 0.53). The skin thickness-assessed using the modified Rodnan skin score (mRSS)-had a significant negative correlation with the reaction size (Rho -0.23; p = 0.003). CONCLUSION: The TST is not sufficiently sensitive for detecting TB infection in SSc patients, albeit a skin induration of ≥ 15 mm indicates a high specificity for tuberculosis infection. A high mRSS resulted in a smaller skin reaction size when using the TST, which has limited utility as a diagnostic for tuberculosis among SSc patients with severe skin thickness. The manuscript was presented as a poster presentation at the Annual European Congress of Rheumatology EULAR 2019 Madrid 12-15 June 2019. (Ann Rheum Dis. 2019;78(suppl 2): abstract FRI0347) http://dx.doi.org/10.1136/annrheumdis-2019-eular.1456.

Skin model for improving the reliability of the modified Rodnan skin score for systemic sclerosis.

BACKGROUND: The gold standard for skin thickness assessment in systemic sclerosis (SSc) is the modified Rodnan skin score (mRSS); however, inter- and intra-rater variation can arise due to subjective methods and inexperience. The study aimed to determine the inter- and intra-rater variability of mRSS assessment using a skin model. METHODS: A comparative study was conducted between January and December 2020 at Srinagarind Hospital, Khon Kaen University, Thailand. Thirty-six skin sites of 8 SSc patients underwent mRSS assessment: 4 times the first day and 1 time over the next 4 weeks by the same 10 raters. No skin model for mRSS assessment was used for the first two assessments, while one was used for the remaining three rounds of assessments. The Latin square design and Kappa statistic were used to determine inter- and intra-rater variability. RESULTS: The kappa agreement for inter-rater variability improved when the skin model was used (from 0.4 to 0.5; 25%). The improvement in inter-rater variability was seen in the non-expert group, for which the kappa agreement rose from 0.3 to 0.5 (a change of 66.7%). Intra-rater variability did not change (kappa remained at 0.9), and the long-term effect of using a skin model slightly decreased by week 4 (Δkappa 0.9-0.7). CONCLUSIONS: Using a skin model could be used to improve inter-rater variation in mRSS assessment, especially in the non-expert group. The model should be considered a reference for mRSS assessment in clinical practice and health education.

Prevalence of peripheral eosinophilia and clinical associations in systemic sclerosis patients.

BACKGROUND: Peripheral eosinophilia (eosinophilia) is observed among systemic sclerosis (SSc) patients. The association between eosinophilia and SSc in terms of pathogenesis remains uncertain. We aimed to determine the prevalence of the clinical, serological, and cytokine associations with eosinophilia in SSc patients. METHODS: A cross-sectional study was conducted among adult SSc patients. We excluded patients having overlap syndrome and other conditions that cause eosinophilia. Investigations into the etiology of eosinophilia were performed on the same study date, including clinical parameters, blood tests for tissue parasites, IgE, interleukin-5, and transforming growth factor-beta. Eosinophilia is defined when the total eosinophil count is > 500 cells/mm3. RESULTS: According to the sample size calculation, 185 patients were enrolled, of whom 57 (30.8%) had eosinophilia. The causes of eosinophilia were based on laboratory indicators without clinical symptoms in 21 cases (10 had a parasitic infection, 9 adrenal insufficiency, and 2 tuberculosis). After excluding suspected causes of eosinophilia, the total prevalence of eosinophilia was 21.9% (95%CI 15.9-29.1). Most of patients (164 cases; 70.6%) had diffuse cutaneous SSc. According to the logistic regression analysis, the factors associated with eosinophilia were being male (OR 3.46), duration of disease increasing every year (OR 1.16), and Raynaud's phenomenon (OR 0.27), while SSc subset, serology (i.e., anti-topoisomerase I, anti-neutrophilic cytoplasmic antibody), inflammatory markers, and cytokine levels were not. CONCLUSIONS: Eosinophilia of unknown causes was detected in 1 in 5 SSc patients, particularly in males with no vasculopathy. Eosinophilia has a nonspecific role vis-à-vis clinical relevance in SSc.

Prevalence of moderate to severe lower urinary tract symptoms in systemic sclerosis.

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.

Prevalence and clinical association of the presence of anti-neutrophilic cytoplasmic antibody in systemic sclerosis.

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.

The prevalence and clinical features associated of hypothyroidism among Thai systemic sclerosis patients.

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.

Clinical Outcomes of Asymptomatic Cardiac Involvement in Systemic Sclerosis Patients After a 2-Year Follow-Up (Extended Study).

BACKGROUND: Asymptomatic cardiac involvement in systemic sclerosis (SSc) has been reported. Long-term follow-up might elucidate the clinical implications of these abnormalities. The aim was to identify the clinical outcomes of asymptomatic cardiac involvement in SSc patients after 2 years of follow-up. METHODS: A cohort study was done at Khon Kaen University, Thailand, on adult patients with SSc who completed the preliminary study. Repeated investigations included electrocardiography, chest radiography, echocardiography, and blood tests for creatine kinase-MB, high sensitivity cardiac troponin-T, and N-terminal prohormone of brain natriuretic peptide. RESULTS: Seventy-four of the 103 patients from the previous study were enrolled. The mean duration of follow-up was 3.1 ± 0.9 years. Five patients developed symptomatic cardiac involvement-all of whom had pulmonary arterial hypertension (PAH). The incidence of symptomatic cardiac involvement for the combined 315 person-years was 1.6 per 100-person-years (95%CI 0.7-3.4). Fourteen patients died resulting in a mortality incidence of 4.4 per 100-person-years (95%CI 4.3-5.4). Persistent cardiac involvement was found in 35 patients for an incidence of 11.1 per 100-person-years (95%CI 8.0-15.5). Two of the patients who had persistent elevated cardiac enzyme developed PAH at a respective 3.7 and 39.4 months after the initial evaluation. None of the clinical parameters were predictive of symptomatic and persistent cardiac involvement. Only male sex was associated with mortality (hazard ratio 3.70; 95%CI 1.22-11.11). CONCLUSIONS: Cardiac involvement in SSc can progress slowly or even be reversed. Based on a previous test, the incidence of symptomatic cardiac involvement after 2 years was low despite its being a persistent involvement. If symptomatic cardiac involvement develops, PAH is the most prevalent symptom.