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.

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.

Is Adjunctive Hyperbaric Oxygen Treatment Alone or with Surgery the Proper Management for Active and Persistent Osteoradionecrosis?

ABSTRACT: This study enrolled patients with stage 3 osteoradionecrosis. There were three treatment modalities: antibiotics, hyperbaric oxygen therapy (HBOT), and surgery. Complete healing was the primary outcome. Multiple logistic regression analysis demonstrated that surgery with HBOT improved wound healing better than HBOT alone.

Associated factors of early-onset pulmonary hypertension and clinical difference between early- and late-onset pulmonary hypertension in Thai systemic sclerosis.

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

Radiation ulcer treatment with hyperbaric oxygen therapy and haemoglobin spray: case report and literature review.

OBJECTIVE: To explore the effectiveness of a combination of hyperbaric oxygen therapy and haemoglobin spray in radiation ulcer treatment. METHOD: We reviewed the available literature and present a case report in which radiation ulcer was treated with a combination of hyperbaric oxygen therapy and haemoglobin spray. RESULTS: After 30 sessions of hyperbaric oxygen therapy (2.4 ATA; 90 minutes each session) and administration of haemoglobin spray, the wounds showed gradual progress towards healing and a good granulating base was achieved. The wounds were closed after two months using a small split thickness skin graft. CONCLUSION: A combination of hyperbaric oxygen therapy and haemoglobin spray was effective as a short course of treatment for radiation ulcers.

Effect of season on clinical outcomes of Thai systemic sclerosis: Analysis of the Thai national healthcare database.

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 performed of patients over 15 in the national database with a primary diagnosis of SSc (ICD-10:M34) covered by the National Health Security Office hospitalized between 2014 and 2018. The seasons were stratified into hot, rainy, and Cool dry seasons.Results: Included were 2480 SSc patients with 3684 admissions: most (64.3%) were female. The respective mean age and median length of stay was 56.9 ± 12.3 years and 3 days (IQR 2-6). The admission rate was highest during the rainy season (1574 visits, 42.7%), followed by the cool dry season (1183 visits, 32.1%) then the hot season (927 visits, 25.2%). During the 13,180 person-days, 1660 SSc patients died resulting in a mortality rate of 12.1 per 100 person-days. The proportion of SSc patients who died in the Cool dry season was significantly higher than in the hot or rainy season (p = .04). Pulmonary involvement in SSc was the most common cause of death in the hot season, which is greater than in the other seasons (p = .004). By comparison, death due to cardiac involvement in SSc was common during the rainy season and cool dry seasons (p = .04).Conclusion: The admission rate among Thai SSc patients was greatest during the rainy season, while mortality was highest during the cool dry season. The most common causes of death were SSc-related irrespective of season, particularly cardiopulmonary involvement.

Clinical characteristics and mortality rate of Thai elderly-onset systemic sclerosis.

OBJECTIVES: To identify the clinical differences and mortality rate between adult and elderly onset systemic sclerosis (SSc). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Dapsone-induced severe cutaneous adverse drug reactions are strongly linked with HLA-B*13: 01 allele in the Thai population.

OBJECTIVES: A previous publication in Chinese leprosy patients showed that the HLA-B*13:01 allele is a strong genetic marker for dapsone-induced drug hypersensitivity reactions, however there are no data describing whether HLA-B*13:01 is a valid marker for prediction of dapsone-induced drug hypersensitivity reactions in other ethnicities or nonleprosy patients. The aim of this study is to investigate whether there is an association between HLA genotypes and dapsone-induced severe cutaneous adverse reactions (SCARs) in Thai nonleprosy patients. PATIENTS AND METHODS: HLA-B genotypes of 15 patients with dapsone-induced SCARs (11 drug reaction with eosinophilia and systemic symptoms, 4 Stevens-Johnson syndrome/toxic epidermal necrolysis), 29 control patients, and 986 subjects from the general Thai population were determined by the reverse PCR sequence-specific oligonucleotides probe. RESULTS: The HLA-B*13:01 allele was significantly associated with dapsone-induced SCARs compared with dapsone-tolerant controls (odds ratio: 54.00, 95% confidence interval: 7.96-366.16, P=0.0001) and the general population (odds ratio: 26.11, 95% confidence interval: 7.27-93.75, P=0.0001). In addition, HLA-B*13:01 associated with dapsone-induced SJS-TEN (OR: 40.50, 95% confidence interval: 2.78-591.01, P=0.0070) and DRESS (OR: 60.75, 95% confidence interval: 7.44-496.18, P=0.0001). CONCLUSION: This study demonstrated an association between HLA-B*13:01 and dapsone-induced SCARs including Stevens-Johnson syndrome/toxic epidermal necrolysis and drug reaction with eosinophilia and systemic symptoms in nonleprosy patients. Moreover, these results suggest that the HLA-B*13:01 allele may be a useful genetic marker for prediction of dapsone-induced SCARs in Thai and Han-Chinese populations.

Effectiveness of add-on therapy with domperidone vs alginic acid in proton pump inhibitor partial response gastro-oesophageal reflux disease in systemic sclerosis: randomized placebo-controlled trial.

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).

Asymptomatic cardiac involvement in Thai systemic sclerosis: prevalence and clinical correlations with non-cardiac manifestations (preliminary report).

OBJECTIVE: To determine the prevalence of asymptomatic cardiac involvement and its correlation with non-cardiac manifestation in Thai SSc patients. METHODS: A cross-sectional study was carried out between January 2012 and June 2013 at Srinagarind Hospital, Khon Kaen University, Thailand, on adult SSc patients without signs or symptoms suggestive of cardiac involvement. We excluded those with overlap syndrome, having serum creatinine >123.8 µmol/l, history of cardiac diseases, any atherosclerosis risk factors and receiving angiotensin-converting enzyme inhibitors. Non-invasive tests related to cardiac involvement were performed, including: echocardiography, ECG, chest X-ray, inflammatory biomarkers, cardiac enzymes and N-terminal prohormone of brain 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.

Inter- and Intra-Observer Reliability of Modified Rodnan Skin Score Assessment in Thai Systemic Sclerosis Patients: A Validation for Multicenter Scleroderma Cohort Study.

BACKGROUND: 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. OBJECTIVE: 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. MATERIAL AND METHOD: 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. RESULTS: 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). CONCLUSION: 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.

Incidence of pulmonary hypertension in patients with systemic sclerosis and no pulmonary symptoms: is annual echocardiographic screening necessary?

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.

Clinical characteristics and mortality in systemic sclerosis: a comparison between early- and late-referred diseases.

OBJECTIVE: To determine if there is any significant difference in the clinical characteristics and mortality between early-and late-referred systemic sclerosis (SSc). MATERIAL AND METHOD: 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. RESULTS: 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 (p < 0.001, 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). CONCLUSION: 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.

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.

Clinical courses and predictors of left ventricular systolic dysfunction in systemic sclerosis: A cohort study.

Background and Objectives: Left ventricular systolic dysfunction (LVSD) is a cardiac involvement that is the leading cause of death among patients with systemic sclerosis (SSc). We aimed to define the clinical course and predictors of LVSD among SSc patients. Methods: We conducted a cohort study among adult patients with SSc who were followed up from 2013 to 2020. Semiparametric Cox regression analysis with robust clustering by cohort identification number was used to evaluate the predictors of LVSD. Results: Among the 3, 987 person-years, LVSD was defined in 35 of 419 SSc patients for an incidence of 0.88 per 100 person-years. The median duration of the disease was 8.5 (interquartile range (IQR) 4.9-12.9) years. Every 1-point increase in the modified Rodnan skin score (mRSS) and salt and pepper skin were strong predictors of LVSD, with a respective adjusted hazard ratio (HR) of 1.05 and 3.17. During follow-up, 26 cases (74.3%) had unimproved LVSD. The strong predictors of the unimprovement of LVSD were every 1-point increase in mRSS (HR 1.05), every 1 mg increase in prednisolone treatment (HR 1.05), and every 1 U/L increase in creatine kinase (CK) (HR 1.001). Mycophenolate treatment was a protective factor against the unimprovement of LVSD in SSc (HR 0.15). Conclusions: LVSD was frequently found in patients with diffuse cutaneous SSc, and in most cases, it remained unimproved during follow-up. High mRSS, steroid use, and high CK levels were predictors of unimproved LVSD, whereas mycophenolate treatment might prevent the progression of LVSD. Steroids should be prescribed with caution in patients with longer disease duration.

The incidence and prevalence of ankylosing spondylitis in Thailand using ministry of public health database.

Ankylosing spondylitis (AS), an inflammatory condition affecting axial and peripheral joints, exhibits varying prevalence worldwide. This study sought to ascertain AS incidence and prevalence in Thailand from 2017 to 2020. Utilizing national databases, individuals aged 18 and above with primary AS diagnoses (ICD-10 code M45) were identified. AS prevalence and incidence were calculated with 95% confidence intervals. The total number of AS patients was 13,292 patients in 2017. The prevalence of AS was 20.4 per 100,000 populations (95% CI 20.0-20.7) in 2017. The number of new AS cases, identified during 2018-2020, was 6784, 6805, and 6791 patients, respectively. The incidences of AS in 2018, 2019, and 2020 were comparable with the incidence of 10.4 (95% CI 10.1-10.6) per 100,000-person-years. The peak age at diagnosis was 50-59 years of age between 2018 and 2020. The number of female patients was 57.8%, 57.0%, and 57.6%, in 2018, 2019, and 2020, respectively. In conclusion, AS was relatively rare among Thais and comparable between males and females. The prevalence and incidence of AS in Thailand were identified by the Thailand Database Ministry of Public Health. The epidemiological profile of AS in Thailand might help to plan better care, workforce needs, and public health budgets.

Clinical outcomes and associated factors with mortality in systemic sclerosis patients with sarcopenia.

BACKGROUND: Despite the high incidence of sarcopenia in systemic sclerosis (SSc) patients, there is currently limited evidence on their outcomes. OBJECTIVES: Our study aimed to determine clinical courses, outcomes, and identify factors associated with mortality in the SSc patients with sarcopenia. METHODS: A historical cohort study was conducted in 180 adult SSc patients diagnosed with sarcopenia according to the criteria of Asian Working Group for Sarcopenia 2019, who were attending the Scleroderma Clinic at Khon Kaen University between July 2019 and November 2021. RESULTS: Forty-one were diagnosed with sarcopenia. A total of 443.8 persons-year, the respective mortality rate for SSc patients with and without sarcopenia was 5.05 and 5.22 per 100-person-years, showing no statistical difference (p = 0.58). Sarcopenia was not a significant mortality risk in SSc patients with a hazard ratio (HR) of 1.34, 95 % CI 0.48-3.75. The survival rate from the baseline evaluation of sarcopenia to the last follow-up of the patients with sarcopenia at 6-, 12-, 18-, and 24-months were 97.6 %, 95.1 %, 92.7 %, and 87.8 %. Hospitalization was the sole factor significantly associated with the mortality risk, with a HR of 14.21 (95 % CI 2.36-85.60). Sarcopenia itself did not appear to be a significant predictor of disease progression, it did contribute significantly to the progression of salt and pepper skin (p=0.01). CONCLUSIONS: The mortality rate of SSc patients with sarcopenia increased after a 2-year follow-up but no difference from non-sarcopenic patients. Once these patients required hospitalization, the mortality risk increased by over 10 times. Further long-term follow-up in a large cohort is suggested.

Outcomes of myocarditis in systemic sclerosis: A 3-year follow-up.

Background and Objectives: The clinical course, the outcomes of myocarditis, and the imaging progression of cardiac magnetic resonance imaging (MRI) in systemic sclerosis (SSc) are still unknown. We aimed at defining changes in cardiac MRI findings, the clinical course, and the outcomes of SSc patients previously defined as having myocarditis by cardiac MRI. Methods: This prospective cohort study included SSc patients, who had previously been diagnosed with myocarditis through cardiac MRI at the Scleroderma Clinic of Khon Kaen University, between 2018 and 2020 and had had annual follow-ups of cardiac MRI for at least 3 years. Data on demographics, clinical characteristics, cardiac MRI findings, treatment regimens, and outcomes were collected. Serial cardiac MRI on a yearly basis was analyzed to assess changes in myocardial involvement over the 3-year period. Results: Ten SSc patients diagnosed with myocarditis via cardiac MRI were included. Most belonged to the diffuse cutaneous subset with a mean age of 58.3±8.6 years and were mildly symptomatic. Initial cardiac MRI findings showed myocardial edema and hyperemia in all patients and eight patients had had pre-existing myocardial scars, suggesting disease chronicity. Treatment for concomitant interstitial lung disease involved steroids with either cyclophosphamide or mycophenolate mofetil in 6 patients. Outcomes of myocarditis were stable, improving, and worsening in 4, 4, and 2 patients, respectively. There was no complete resolution of the cardiac MRI indices for myocarditis, and none had had major cardiac events. Conclusion: Although SSc myocarditis on cardiac MRI may improve or show stability, the changes remained persistent. Among patients with SSc and mildly symptomatic myocarditis, the efficacy of steroids and immunosuppressive therapy is inconclusive. Over a 3-year follow-up, the prognosis had been acceptably good with no cardiac events.

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.

Pericardial fluid profiles of pericardial effusion in systemic sclerosis patients.

BACKGROUND: Cardiac involvement is one of clinical presentations in systemic sclerosis (SSc). The pericardial fluid profile and the causes of pericardial effusion were our focus. OBJECTIVES: To demonstrate the characteristics and causes of pericardial effusion in SSc. METHODS: 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. RESULTS: 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 ratio <0.6. Most of the cases (87.5%) had a fluid-serum total protein ratio <0.5. 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. CONCLUSIONS: 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.