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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.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/mmAccording 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.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.

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.

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.

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.

Proton pump inhibitor (PPI) twice daily dosing is a standard therapy for gastroesophageal reflux disease (GERD) in systemic sclerosis (SSc) but there is no data on its response rate or the predictors of PPI-partial response GERD. Aims were to determine the prevalence of PPI-partial response GERD in SSc and to define its predictors. A prospective study was conducted in SSc patients with GERD. The patients were treated with omeprazole 20 mg bid for 4 weeks. The severity of symptom-grading by visual analogue scale (VAS) and frequency of symptoms by frequency scale for symptoms of GERD (FSSG) were assessed at baseline and 4 weeks after treatment. PPI-partial response GERD was defined as less than 50% improvement in the VAS for severity of symptom as well as acid reflux score by FSSG after treatment. According to the sample size calculation, 243 SSc-GERD patients were enrolled; of whom 166 (68.3%) had the diffuse cutaneous SSc. PPI-partial response GERD was found in 131 SSc patients (prevalence 53.9%; 95%CI 47.4–60.3). The multivariate analysis revealed that esophageal dysphagia was an only predictor the PPI-partial response GERD (OR 1.82; 95%CI 1.01–3.29) while neither SSc subset nor severity of skin tightness were significantly associated with PPI-partial response GERD. Half of the SSc patients were PPI-partial response GERD. Esophageal dysphagia was the only predictor of PPI-partial response GERD in SSc patients. Screening for dysphagia before starting GERD treatment is helpful for assessment the risk of PPI refractoriness GERD in SSc patients.

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 3 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.

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 2) 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.

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.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.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).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.

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.

Objectives: We aimed to determine rate of admission, mortality rate, and causes of death in systemic sclerosis (SSc) patients after stratifying by season.Method: A cross-sectional analysis was perf...

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 (dcSSc) 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 (Lubeck, 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 dcSSc 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.

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

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.

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.

To identify the clinical differences and mortality rate between adult and elderly onset systemic sclerosis (SSc).We conducted a historical cohort study of SSc patients during January 2007-December 2011. The SSc patients were 60 and over classified as elderly onset SSc. Cox regression analysis was used to estimate the probability of survival and for assessing the factors associated with mortality.The medical records of 350 SSc patients were reviewed; 53 (15.1%) had elderly onset SSc. According to the multivariate analysis, elderly onset SSc has a higher WHO functional class, more frequent weakness, more frequent hyperCKaemia, and less pulmonary fibrosis than adult onset SSc (p=0.004, 0.02, 0.02, 0.02, respectively). The incidence of mortality was 3.8 per 100 person-year with a median survival rate of 15.9 years (95%CI 12.4-17.3). The mortality rate of elderly SSc onset was significantly higher than that of adult SSc onset (HR 5.71; 95%CI 3.54-9.20). The median survival of elderly and adult onset SSc was 4.9 years and 16.1 years, respectively. The Cox regression analysis indicated that presence of digital ulcer and tendon friction rub had a respective HR of 7.39 (95%CI 1.28-42.60) and 37.23 (95%CI 2.10-659.09) for predicting mortality of elderly onset SSc.Myopathy and limitation of physical activity were frequently found among elderly onset SSc over against pulmonary involvement than in adult onset SSc. Mortality of elderly onset SSc was 5.7 times higher, and median survival was 11 years shorter, than adult onset SSc.

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

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.To define the clinical differences between SSc, positive versus negative, for ATA.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.Two hundred and ninety-four SSc patients were included (female : male 2.5 : 1). The majority (68.6%) were the diffuse cutaneous SSc subset (dcSSc). ATA was positive in 252 patients (85.7%), among whom 71.7% had dcSSc and 28.2% limited cutaneous SSc (lcSSc). 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 dcSSc 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 lcSSc sufferers who were ATA negative than those who were ATA positive (90.5% vs. 56.9%, P = 0.005).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 dcSSc and a lower frequency of RP in lcSSc.

Background Annual echocardiographic screening has been recommended for early detection of pulmonary hypertension (PHT) in systemic sclerosis (SSc) patient; however, the cost benefit for cases with no pulmonary symptoms is not well established. Objectives The objective of this study was to determine the incidence and clinical predictors of echocardiographic diagnosis of PHT in "asymptomatic" SSc patients. Methods A historical cohort of adult SSc patients who underwent echocardiographic screening for PHT at least twice in Khon Kaen University, Thailand, during January 1, 2005, to December 31, 2011, was included. Pulmonary hypertension was defined by an estimate of right ventricular systolic pressure of greater than 40 mm Hg. Results A total of 143 medical records were reviewed (female-to-male ratio = 1.7:1). The majority had the diffuse subset (94 cases; 69.6%). Seventy-five (52.4%) were without pulmonary symptoms throughout the follow-up period. Of the 403.1 person-years under observation in the asymptomatic group, only 1 had pulmonary arterial hypertension confirmed by right-sided heart catheterization. The incidence of PHT among such SSc patients was 0.2 per 100 person-years (95% confidence interval [CI], 0.006-0.01 per 100 person-years). The disease duration at the time of PHT detection by echocardiography was 5.1 years. The declining functional class (FC) led to the higher incidence of PHT, with incidence of PHT in those who had declining FC I to FC II and FC I to FC III of 2.1 (95% CI, 0.4-6.0) and 4.2 (95% CI, 0.1-23.3), respectively. Conclusions The incidence of echocardiography-diagnosed PHT among Thai with SSc, and no pulmonary symptoms was low. Annual echocardiography is less beneficial among such patients; repeated echocardiography should instead be performed on those with a declining FC.

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.

Renal involvement in scleroderma is life-threatening. Early detection of a deterioration of the glomerular filtration rate (GFR) is needed to preserve kidney function.To (A) determine the correlation between (1) estimated GFR (eGFR) using 4 different formulae and (2) measured GFR (mGFR) using isotopic renal scan in Thai patients with scleroderma with normal serum creatinine and (B) to define the factors influencing eGFR.A cross-sectional study was performed in adult Thai patients with scleroderma at Srinagarind Hospital, Khon Kaen University, between December 2013 and April 2015. GFR was measured using the gold standard Tc-99m DTPA (Tc-99m diethylenetriaminepentaacetic acid) renal scan. We compared the latter with the eGFR, calculated using the Cockroft-Gault formula, Modification of Diet in Renal Disease (MDRD), Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation and creatinine clearance equation.A total of 76 patients with scleroderma (50 women and 26 men) with median age 54.8 years (interquartile range: 47.4 to 58.9) were enrolled. Mean disease duration was 5.6 ± 4.5 years. Median value of mGFR was 100.1 ± 27.6mL/minute/1.73m². There was a correlation between mGFR from the Tc-99m DTPA renal scan and the eGFR using the Cockroft-Gault formula, MDRD and CKD-EPI equation (P = 0.01,0.001 and0.001, respectively), but no correlation with eGFR using the creatinine clearance equation (P = 0.27). Body weight, prednisolone use and systolic blood pressure (SBP) had a negative association with mGFR (P = 0.01, 0.01 and 0.007, respectively). After multivariate analysis, SBP was the only clinical parameter that influenced mGFR (P = 0.03).The Cockroft-Gault formula, MDRD study equation and CKD-EPI were useful formulae for assessing GFR in Thai patients with scleroderma. Higher SBP was associated with a lower GFR.

Assessment of the severity of skin tightness by the modified Rodnan skin score (mRSS) for systemic sclerosis (SSc) has been found feasible, valid, and reliable. Despite being a major clinical outcome, it has not yet been validated by Scleroderma Research Group.To (a) determine the inter-observer variability vis-à-vis mRSS assessment by members ofthe Scleroderma Research Group before and after mRSS-assessment training by an experienced rheumatologist and (b) determine intra-observer variability.Between June and August 2013, we conducted a descriptive study of Thai adult SSc patients and all rheumatologists in the Scleroderma Research Group at Srinagarind Hospital, Khon Kaen University, Northeast Thailand. Eleven rheumatologists assessed the mRSS of 22 SSc patients three times (i.e., before and after training, and eight weeks after training). The intra-class correlation coefficient (ICC) and its 95% CI were estimated at week 8 after training.The mean and standard deviation (SD) of mRSS for inter-observer variability analysis was slightly decreased from before training, after training (by an experienced rheumatologist), and at week 8 after training (17.3 ± 11.9, 16.5 ± 11.1, and 16.2 ± 10.3, respectively). Intra-observer variability had moderate agreement before training (ICC 0.59; 95% CI 0.38-0.78), which increased to good agreement after training and at week 8 after training (ICC 0.60; 95% CI 0.42-0.76 vs. 0.68; 95% CI 0.53-0.82, respectively).Inter-observer variability for mRSS assessment decreased after training and the reduction persisted for eight weeks after training. The ICC rose from moderate agreement at baseline to good agreement at the end of the study. The mRSS assessment by members of the Scleroderma Research Group was reliable.

Infection is a common cause of death in systemic sclerosis (SSc) but despite immunosuppressant therapy, there are few reports of opportunistic infection.To estimate the incidence of infection, to determine the etiologic organism, and to assess the risk factors for infection among Thai SSc patients.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.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.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.

Objectives Twice-daily dosing of proton pump inhibitor (PPI), the standard therapy for gastro-oesophageal reflux disease (GERD), is an effective therapy for GERD in SSc. The aim of this study was to compare the efficacy of omeprazole in combination with domperidone vs in combination with algycon in reducing the severity and frequency of reflux symptoms of PPI partial response (PPI-PR) GERD in SSc. Methods Adult SSc patients having PPI-PR GERD were randomly assigned to receive domperidone plus algycon placebo or algycon plus domperidone placebo in a 1:1 ratio plus omeprazole for 4 weeks. The assessment included severity of symptom grading by visual analogue scale, frequency of symptoms by frequency scale for symptoms of GERD and quality of life (QoL) by EuroQol five-dimensions questionnaire scoring. Results One hundred and forty-eight SSc-GERD patients were enrolled, of whom 88 had PPI-PR. Eighty cases were randomized for either domperidone (n = 38) or algycon (n = 37) therapy. The majority in both groups had the diffuse SSc subset. At the end of the study, no significant difference in symptom grading was found between groups. After treatment and compared with baseline, the severity of symptoms, frequency scale for symptoms of GERD and QoL significantly improved in both groups. Five (13.2%) and 8 (21.6%) respective cases in the domperidone and algycon groups did not respond. Conclusion The prevalence of PPI-PR GERD is common. Domperidone and algycon are equally effective treatments in combination with omeprazole. However, ∼17% of patients were non-responsive, so the effectiveness of domperidone, algycon and PPI combination therapy should be further investigated. Trial registration https://clinicaltrials.gov (NCT01878526).

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.

To determine if there is any significant difference in the clinical characteristics and mortality between early-and late-referred systemic sclerosis (SSc).An historical cohort study was performed among referred-SSc patients at Srinagarind Hospital between January 2006 and December 2010. 'Early referrals'occurred during the edematous phase while 'late referrals' occurred after that.Forty two percent of the SSc cases (229 of 543) were referred; 108 (47.2%) were early-referrals. Early referrals were for proper management (49.1%) and diagnosis (41.7%), whereas the majority of late referrals (79.3%) were for proper management, followed by additional investigations (10.7%). The respective median duration of disease at referral between early and late was 3.7 (IQR 2.6-5.6) and 20.7 months (IQR 12.2-37.4). Joint contracture, cardiac involvement and pulmonary fibrosis presented more frequently among late-referrals (p0.001, p = 0.03 and p = 0.04, respectively). The respective mortality rate among early- vs. late-referrals was 15.1 (95% CI 10.0-21.8) vs. 23.0 (95% CI 15.8-32.3) per 100 person-year Two-thirds of deaths were associated with the disease, pulmonary fibrosis being most common among both early- and late-referrals (50 and 42.7%, respectively).The number of early vs. late referrals was comparable and cardiopulmonary involvement and joint contracture were common presentations in late-referrals. Late-referral was associated with high mortality commonly from pulmonary fibrosis.

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 Sj€ 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.

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.

Cardiac involvement is one of clinical presentations in systemic sclerosis (SSc). The pericardial fluid profile and the causes of pericardial effusion were our focus.To demonstrate the characteristics and causes of pericardial effusion in SSc.A descriptive retrospective study was performed on SSc patients with symptomatic pericardial effusion at Srinagarind Hospital, Khon Kaen University, Thailand, between January 1, 2000 and December 31, 2010. We excluded pericardial fluid detected by screening echocardiography.Thirty medical records of SSc patients with pericardial effusion were reviewed. The respective mean age and median duration of disease at the time of effusion detection was 52.2±10.8 years and 11.4 months (IQR 0.03-59.1). The female to male ratio was 3.3:1. The most common signs and symptoms included tachycardia (65%) and right-sided heart failure (30%). One patient was diagnosed with cardiac tamponade due to tuberculous pericarditis. Pericardiocentesis and fluid analysis were performed in 9 patients, all of whom had pericardial fluid lactate dehydrogenase (LDH)200 U/L and a fluid-serum LDH ratio0.6. Most of the cases (87.5%) had a fluid-serum total protein ratio0.5. The median white blood cell (WBC) count was 10 cell/mm3 (IQR 6-830), one-third of which was predominated by mononuclear cells. The pericardial biopsy in 8 patients revealed 4 pericardial fibrosis, 2 non-specific inflammation and 2 granuloma.The pericardial fluid profile for SSc was similar to the exudative profile of pleural fluid. Cardiac tamponade was rare. Most pathological findings of pericardial tissue were associated with fibrosis related to the disease itself.

Avascular necrosis (AVN) has been reported in systemic lupus erythematosus (SLE) and most SLE patients suffer from this problem.To study the prevalence of AVN in Thai SLE patients and to determine the risk factors for developing AVN.A retrospective study was performed, between January 1, 1995 and August 31, 2005, on patients over 15 years of age in Khon Kaen, Thailand.The medical records of 736 SLE patients were reviewed. The female to male ratio was 15.4:1. The prevalence of AVN was 8.8%. The average age at the time of AVN detection was 27 years (range, 18-54) and the average duration of disease 69 months (range, 12-112). All cases were AVN of the hip joint. The factors correlated with AVN included: long duration of disease, history of previous septic arthritis in the ipsilateral hip to the AVN development, hematological involvement, gastrointestinal involvement, arthritis and cutaneous vasculitis. After regression analysis, hematological involvement and long duration of disease were associated with AVN with a respective odds ratio of 3.13 (95% CI 1.13-8.54) and 1.01 (95% CI 1.00-1.02). Neither high-dose steroid nor antimalarial treatment were correlated with AVN in our study and 4.6% (n = 3) of patients had never received steroid therapy during the follow-up period.Prevalence of symptomatic AVN was 8.8% in our SLE patients. A longer duration of disease and hematological involvement were associated with AVN development.

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

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.Our objective was to identify the causes of death and the factors predictive of mortality and to calculate the survival rate among SLE patients.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.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 onset50 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.

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.To study the clinical features and natural course of UA in Thai patients.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.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).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.

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.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.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%).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.

Tuberculous septic arthritis is difficult to diagnose. A retrospective analysis was done on patients over 15 years of age who attended Srinagarind Hospital, Khon Kaen, Thailand, between January 1, 1997 and December 31, 2006, whose synovial fluid culture was positive for Mycobacterium tuberculosis. The medical records of 77 patients were reviewed; one-third were in their sixth decade. Comorbid disease was found in 33 cases (42.9%), with systemic sclerosis being the most common (9 cases) followed by diabetes mellitus (5 cases) and chronic kidney disease (5 cases). Chronic monoarthritis was the most common presentation (34 cases) followed by acute monoarthritis (20 cases). More than half of the polyarticular involvements were disseminated tuberculosis. The knee was the most commonly affected joint (36.4%). Sixty percent had delayed diagnosis due to an incorrect diagnosis. Abnormal chest radiography and blood eosinophilia were found in 40 and 57.3% of cases, respectively. Synovial fluid and synovial tissue staining for acid-fast bacteria were positive in 30 and 40% of cases, respectively. A caseous granuloma was present in 57.5% of cases and non-specific synovitis in 12%. Sixty-three percent had bone erosions. Tuberculous septic arthritis should be considered in patients who present with acute or chronic monoarthritis, and who have an abnormal chest radiograph or eosinophilia. Polyarticular involvement was commonly related to having disseminated tuberculosis and may indicate systemic involvement of tuberculous infection.

Micro-vascular thrombus is a common pathological finding in pulmonary artery hypertension. The association between plasma D-dimer, a marker of thrombus formation, and pulmonary artery hypertension (PAH) in patients with systemic sclerosis is unknown.To assess the correlation of the level of plasma D-dimer and pulmonary artery pressure in patients with systemic sclerosis.One hundred and twenty nine patients with systemic sclerosis between 19 and 75 years of age (mean, 48 +/- 11.3) entered the study. Plasma D-dimer was determined using immunoturbidimetric assay (D-dimer plus, Dade Behring Inc., Newark, USA). Pulmonary artery pressure was estimated by Doppler echocardiography. PAH was considered present if the Doppler echocardiography-estimated right ventricular systolic pressure (RVSP) exceeded 36 mmHg.Forty-seven patients (36.4%) had PAH according to Doppler echocardiography including 32 (68.1 %) mild (RVSP, 36-45 mmHg), nine (19.1%) moderate (RVSP, 46-55 mmHg), and six (12.8%) severe PAH (RVSPor = 56 mmHg). No significant correlation was found between plasma D-dimer and RVSP (r = 0. 02, p = 0. 82).The present study demonstrated that the D-dimmer level is not associated with the level of pulmonary artery pressure in patients with systemic sclerosis, indicating that microvascular thrombosis may not play a significant role in the pathogenesis of PAH in patients with systemic sclerosis.

Tuberculosis, caused by Mycobacterium tuberculosis is a common infection both in immunocompromised and normal hosts. Its clinical manifestation can by divided as pulmonary and extrapulmonary form. Pyomyositis caused by M. tuberculosis is extremely rare. The authors report 2 patients, one with underlying dermatomyositis, and the other with polymyositis. The diagnosis was delayed according to nonspecific symptoms and masking effect of steroid therapy, which led to complications. Microscopy and culture of the pus confirmed the diagnosis. Surgical drainage was done and antituberculous therapy was given. The patient with dermatomyositis was complicated by drug induced hepatitis and died but the other was cured. Tuberculous pyomyositis should be considered in patients who are immunocompromised hosts.

Salmonella septic arthritis is an infrequent infectious disease but can cause progressive joint destruction resulting in disability. The authors retrospectively reviewed cases with culture proved Salmonella septic arthritis in Srinagarind Hospital, Khon Kaen from 1994 to 2000. There were 23 episodes in 16 cases; all had underlying diseases and a history of steroid abuse or steroid and immunosuppressive therapy. Systemic lupus erythematosus was the most commonly found underlying disease (56%). Salmonella group D and group B were isolated in 13 and 3 cases. Most first episodes had acute onset of monoarthritis. The antibiotics used as initial treatment of the first episodes were beta lactam, cotrimoxazole or quinolones. There were 8 cases with disabled sequelae and 7 cases with relapse. For 13 evaluable first episodes, relapse occurred in 3 cases in the cephalosporin/penicillin and 4 cases in the cotrimoxazole treated group but none in the quinolones. Six relapse cases were treated successfully with quinolones as well as one with cotrimoxazole. Although 5 relapse cases treated with quinolones had previous progressive joint destruction or avascular necrosis, there was no further joint damage after re-treatment with quinolones. In conclusion, quinolones were more effective than beta-lactams and cotrimoxazole for the treatment of Salmonella septic arthritis to prevent relapse and progressive joint destruction.