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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.We aimed to compare the difference between the current and past (pre-disease onset) fingerprints of SSc patients.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.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.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.

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.

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.

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

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.

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.

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

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.

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.

Background. Myocarditis is reported in systemic sclerosis (SSc); however, treatment options and outcomes are limited. Our objective was to define cardiac outcomes after moderate-dose steroid therapy in SSc patients with myocarditis. Method. An open-label study was conducted among SSc patients with myocarditis—as defined by cardiovascular magnetic resonance (CMR), disease onset p = 0.03 ), higher heart rate at baseline ( p = 0.049 ) and week 24 ( p = 0.04 ), lower left ventricular (LV) and right ventricular (RV) stroke volume at baseline ( p = 0.002 and p = 0.01 ) and week 24 ( p = 0.01 and p = 0.02 ), and lower LV and RV cardiac output at week 24 ( p = 0.01 and p = 0.01 ). Four cases died during follow-up (3 due to cardiac complications, 1 due to renal crisis). The two who died from heart failure had very high NT-prohormone-brain natriuretic peptide (NT-proBNP) and impaired LV ejection fraction (LVEF), and the one who died from arrhythmia had very high sensitivity of cardiac Troponin-T (hs-cTnT). Conclusions. Moderate-dose steroid therapy may improve myocarditis in SSc. A proportion of patients died due to cardiac complications during treatment, particularly those with high hs-cTnT, high NT-proBNP, and impaired LVEF. This trial is registered with NCT03607071.

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 Eosinophilia has been reported in systemic sclerosis (SSc) and localized scleroderma, so it might be part of the immune response in the pathogenesis of the disease. Objectives To determine the prevalence and clinical associations with peripheral eosinophilia in Thai SSc patients. Methods A cross-sectional study was conducted among Thai adult SSc patients, followed up at the Scleroderma Clinic, Khon Kaen University, Thailand, between November 1, 2016 and November 30, 2017. We excluded patients who had clinical overlap with other connective tissue disease, coexisting with localized scleroderma, eosinophilic fasciitis, or eosinophilia myalgia syndrome, and other diseases that cause eosinophilia. Peripheral eosinophilia is defined when total eosinophil count (TEC) is greater than 500 cells/mm3. Clinical, laboratory tests for tissue parasite, cytokines, and others for evaluation the cause of eosinophilia were done on the study date. Results A total of 185 SSc patients were enrolled. Fifty-seven cases (10.1%) were peripheral eosinophilia of which 21 had the causes of eosinophilia identified by laboratory without clinical symptoms (viz., 9 adrenal insufficiency, 2 tuberculosis, and 10 parasitic infection). The total prevalence of the unknown causes of peripheral eosinophilia in SSc was 21.9% (95%CI 15.9-29.1) (36 of 164 cases). Five of the patients had TEC above 1500 cells/mm3. Of the 164 SSc patients, the majority (70.6%) had diffuse cutaneous SSc, and the female to male ratio was 2.3:1. The respective median age and median duration of the disease was 57.0 years (IQR 52.2-63.9) and 6.5 years (IQR 2.9-10.5). According to a multivariate analysis, being male and duration of disease increasing every year were significantly associated with peripheral eosinophilia in SSc patients (OR 3.46 (95%CI 1.11-10.73) and 1.16 (95%CI 1.03-1.30), respectively), while Raynaud’s phenomenon had a significantly negative correlation with peripheral eosinophilia in SSc (OR 0.27: 95%CI 0.09-0.84). Other parameters—such as SSc subset, severity of skin tightness, serology, cytokines (transforming growth factor-β, interleukin-5)—were not correlated with peripheral eosinophilia. Conclusion Peripheral eosinophilia of unknown cause can be detected 1 in 5 SSc patients. The factors associated with peripheral eosinophilia are longer disease duration and being male while vasculopathy has a negative association. References [1] Falanga V, Medsger TA. Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis. J Am Acad Dermatol. 1987Oct;17(4):648–56. [2] Giordano M, Ara M, Valentini G, Chianese U, Bencivenga T. Presence of eosinophilia in progressive systemic sclerosis and localized scleroderma. Arch Dermatol Res. 1981;271(4):411–7. [3] Tefferi A, Patnaik MM, Pardanani A. Eosinophilia: secondary, clonal and idiopathic. Br J Haematol. 2006Jun;133(5):468–92. [4] Mejia R, Nutman TB. Evaluation and differential diagnosis of marked, persistent eosinophilia. Semin Hematol. 2012Apr;49(2):149–59. [5] Kargili A, Bavbek N, Kaya A, Kosar A, Karaaslan Y. Eosinophilia in rheumatologic diseases: a prospective study of 1000 cases. Rheumatol Int. 2004Nov;24(6):321–4. [6] Gustafsson R, Fredens K, Nettelbladt O, Hallgren R. Eosinophil activation in systemic sclerosis. Arthritis Rheum. 1991Apr;34(4):414–22. Acknowledgement The Scleroderma Research Group and Faculty of Medicine, Khon Kaen University (Grant Number GR60101) Disclosure of Interests None declared

Background The tuberculin skin test (TST) is a screening tool for detection of occult or remote tuberculosis infection. Although skin tightness is a classic manifestation in systemic sclerosis (SSc), there are no reports vis-a-vis any limitation of the TST in SSc patients nor a definition on the cut-off for a positive TSTtest size for a diagnosis of tuberculosis. Objectives Our aims were to determine (a) the indurated reaction size of the TST; (b) the cut-off size for the indurated TST; and, (c) the sensitivity and specificity of the test for the diagnosis of tuberculosis in SSc patients. Methods A cross-sectional study was conducted among Thai adult SSc patients, followed up at the Scleroderma Clinic, Khon Kaen University, Thailand between November 1, 2016 and November 30, 2017. The TST was performed using 0.1 ml purified protein derivatives (PPD) injected intradermally (Figure 1), and interpreted 72 hours after testing. Results A total of 168 SSc patients were enrolled (female to male ratio = 1.8:1). The median age and duration of disease was 57.2 and 6.4 years, respectively. The majority (71.8%) was the diffuse cutaneous SSc subset. Seventeen cases (10.1%) were defined as tuberculosis infection. All of the patients had a history of BCG vaccination at birth. An indurated skin reaction size TST of 20 U/L had a high specificity for tuberculosis (99.3%: 95%CI 96.4-100) (Kappa 0.86; p=0.03). The modified Rodnan skin score (mRSS) had a significant negative correlation with the indurated skin reaction size (Rho -0.23; p=0.003). While other clinical parameters—such as BMI, SSc subset, serum albumin level, steroid or immunosuppressant use—were not correlated with the TST result (p=0.06, 0.14, 0.09, 0.23 and 0.89, respectively). Conclusion Indurated skin ≥ 20 mm indicated a high specificity for tuberculosis infection in SSc patients with history of BCG vaccination. The high mRSS resulted in a smaller skin reaction size when using the TST. The TST is thus less useful as a diagnostic tool for tuberculosis among SSc patients, especially among those with severe skin tightness. References [1] Foocharoen C, Nanagara R, Foocharoen T, Mootsikapun P, Suwannaroj S, Mahakkanukrauh A. Clinical features of tuberculous septic arthritis in Khon Kaen, Thailand: a 10-year retrospective study. Southeast Asian J Trop Med Public Health. 2010 Nov;41(6):1438–46. [2] Rose DN, Schechter CB, Adler JJ. Interpretation of the tuberculin skin test. J Gen Intern Med. 1995 Nov;10(11):635–42. [3] Jamil B, Qamruddin S, Sarwari A, et al. An assessment of Mantoux test in the diagnosis of tuberculosis in a BCG-vaccinated, tuberculosis endemic area. Journal of Infectious Disease Parkistan. 2008;17(1):18–22. [4] Liam C, Lim K, Wong C. Changes in serum proteins, erythrocyte sedimentation rate and Mantoux tuberculin skin test reactivity in active tuberculosis. JUMMEC. 1998;3(1):47–53. [5] Wang L, Turner MO, Elwood RK, Schulzer M, FitzGerald JM. A meta-analysis of the effect of Bacille Calmette Guerin vaccination on tuberculin skin test measurements. Thorax. 2002 Sep;57(9):804–9. [6] Araujo Z, de Waard JH, de Larrea CF, Borges R, Convit J. The effect of Bacille Calmette-Guerin vaccine on tuberculin reactivity in indigenous children from communities with high prevalence of tuberculosis. Vaccine. 2008 Oct 16;26(44):5575–81. [7] Farhat M, Greenaway C, Pai M, Menzies D. False-positive tuberculin skin tests: what is the absolute effect of BCG and non-tuberculous mycobacteria? Int J Tuberc Lung Dis. 2006 Nov;10(11):1192–204. [8] Abdel-Samea S, Ismail Y, Fayed S, Mohammad A. Comparative study between using QuantiFERON and tuberculin skin test in diagnosis of Mycobacterium tuberculosis infection. EJCT. 2013;62:137–43. Acknowledgement The Scleroderma Research Group and the Faculty of Medicine, Khon Kaen University (Grant Number GR60101) Disclosure of Interests None declared

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.

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

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).

Background: The major cause of death in systemic lupus erythematosus (SLE) is due to the disease activityitself or infection. It is uncertain whether the treatment approach during the last decade prolonged survival inSLE.Objective: Our objective was to identify the causes of death and the factors predictive of mortality and to cal-culate the survival rate among SLE patients.Method: We conducted a retrospective study of SLE patients followed up at Srinagarind Hospital, Khon KaenUniversity, Thailand, between January 1, 1996 and August 31, 2005. Cox regression analysis was used to esti-mate the probability of survival and assessing factors associated with death. The medical records of 749 SLEpatients were reviewed; 66 patients died during the follow-up period.Results: The mortality rate was 1.2 per 100 person-years. The 5- and 10-year survival rate among our SLEpatients was 93% and 87%, respectively. The mean age at death was 34.08 11.75 years and the median dis-ease duration was 48 (1–336) months. One-third of the cases were referred from a local hospital more than1 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 neuropsychiat-ric lupus. Factors predictive of mortality included: (i) major organ flare more than four times per year; (ii)age at onset > 50 years; (iii) high-dose steroid use and/or immunosuppressive therapy at onset and within2 weeks prior to death; and (iv) concomitant diabetes mellitus. Protective against mortality was antimalarialuse.Key words: cause of death, lupus, mortality, survival rate, systemic lupus erythematosus.

Aims: Pulmonary arterial hypertension (PAH) is a major complication and cause of death in systemic sclerosis (SSc). Our objective was to identify the predictive factors of mortality and the 2-year survival rate among Thai sufferers of PAH-SSc. Methods: An historical cohort study was performed among PAH-SSc patients followed up at Srinagarind Hospital, Thailand, between January 2005 and December 2008. Kaplan-Meier and Cox regression analyses were used to estimate the probability of survival and to assess the significant factors associated with death. Results: Pulmonary arterial hypertension was recognized in 60 patients using ECHO criteria, right ventricular systolic pressure (RVSP) > 35 mmHg. Two-thirds of the patients were female, > 50 years of age, with the dSSc subtype. Twenty patients (33.3%) died: the mortality rate was 15.6% per 100 person-years. The respective 1-, 2-, 3- and 4-year survival rate was 86.1%, 71.3%, 64.6% and 53.9%. The majority (85%) died without any specific treatment for PAH. Using univariate analysis, the mortality risk was associated with: the WHO functional class (FC) III (HR = 27.82), visceral organ involvement (HR = 5.14), myositis (HR = 3.14), esophageal dysmotility (HR = 3.08) and pericarditis (HR = 2.84). Using Cox regression, the only predictor of death was FCIII. The causes of death in PAH-SSc were related to PAH (60%), infection (30%) and acute renal failure (10%). Conclusion: Up to one-third of Thai sufferers of PAH-SSc died within 2 years of PAH diagnosis, without any specific treatment being given. Increased mortality risk was found in SSc patients who had FCIII and visceral organ involvement.

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.