Predictors and survival of cardiomyopathy determined by echocardiography in Thai patients with early systemic sclerosis: an inception cohort study.

Available data including the incidence, predictors and long-term outcome of early systemic sclerosis patients associated with suspected cardiomyopathy(SSc-CM) is limited. Therefore, we aimed to study the incidence, predictors and survival of SSc-CM. An inception cohort study was conducted for early SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, Thailand, from January 2010 to December 2019. All patients were determined for clinical manifestations and underwent echocardiography and HRCT at enrollment and then annually. SSc-CM was determined and classified using echocardiography. 135 early SSc patients (82 female,108 DcSSc) were enrolled. With the mean follow-up period of 6.4 years, 32 patients developed SSc-CM. The incidence of SSc-CM was 5.3 per 100-person years. The multivariate Cox regression analysis showed that baseline anti-topoisomerase I-positive (Hazard ratio[HR] 4.86, p = 0.036), dysphagia (HR 3.35, p = 0.001), CK level ≥ 500 U/L(HR 2.27, p = 0.045) and low oxygen saturation (HR 0.82, p = 0.005) were predictors of SSc-CM. The survival rates after SSc-CM diagnosis at 1, 5 and 10 years were 90.3%, 73.1%, and 56.1%, respectively. In this study cohort, the incidence of SSc-CM was 5.3 per 100-person years, and tended to have low survival. The presence of anti-topoisomerase I antibody, dysphagia, CK level ≥ 500 U/L, and low oxygen saturation were independent baseline predictors for developing SSc-CM.

Elevated baseline serum creatine kinase in Thai early systemic sclerosis patients is associated with high incidence of cardiopulmonary complications and poor survival: an inception cohort study.

BACKGROUND: Inception cohort data regarding the incidence of cardiopulmonary complications in early systemic sclerosis (SSc) patients comparing those with and without elevated baseline creatine kinase (CK) are limited. This study aimed to compare the incidence of cardiopulmonary complications and survival between the two subgroups. METHODS: We used an inception cohort study of early SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, Thailand, from January 2010 to December 2019. All patients were assessed for clinical manifestations and CK levels and underwent echocardiography and HRCT at the study entry and annually thereafter. RESULTS: A total of 144 SSc patients (84 female, 115 diffuse cutaneous SSc (DcSSc)) with a mean disease duration of 11.9 ± 9.2 months were enrolled. At cohort entry, their mean ± SD CK levels were 364.3 ± 598.0 U/L. The participants were then divided into two subgroups: (i) 29 SSc with elevated CK (baseline CK ≥ 500 U/L); (ii) 115 SSc with non-elevated CK. At enrollment, the elevated CK group was characterized by a higher proportion of male gender, DcSSc subtype, arthritis, and weakness; shorter disease duration; and higher MRSS compared with non-elevated CK. At the last visit, with a mean ± SD follow-up duration of 6.2 ± 2.7 years, the elevated CK group showed a higher cumulative prevalence of weakness, dysphagia, LVEF < 50%, and suspected myocardial disease; higher incidence of LVEF < 50%, suspected myocardial disease, and ILD; and shorter survival time. CONCLUSION: It was found in our study cohort that elevated baseline serum CK in early SSc, of which majority were DcSSc subtype, is associated with more severe clinical presentation, higher incidence of cardiopulmonary complications, and shorter survival time compared with the non-elevated CK subgroup. Key Points • In early SSc patients, elevated baseline serum creatine kinase was confirmed to be associated with a high incidence of cardiac and ILD complications, and poor long-term survival time. • Careful evaluation of baseline serum CK levels in all early-diagnosed SSc patients is crucial in general clinical practice.

Incidence, predictors, and survival of pulmonary hypertension determined by echocardiography in Thai patients with early systemic sclerosis (SSc): inception cohort study.

OBJECTIVES: We investigated the incidence, predictors, and survival of pulmonary hypertension (PH) determined by Doppler echocardiography in Thai patients with early SSc (systemic sclerosis), in which the majority were diffuse cutaneous SSc (DcSSc) with anti-topoisomerase I-positive. METHODS: We used an inception cohort study of patients with early SSc seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital. All patients were assessed for clinical data and underwent Doppler echocardiography at the study entry and then annually. RESULTS: A total of 133 patients (81 female, 106 DcSSc, 103 anti-topoisomerase I-positive) with a mean disease duration of 11.9 months were recruited. During the mean observational period of 4.2 years, 14 patients developed PH concurrent with ILD and 6 with left heart disease. The incidence rate for the development of PH was 3.95 per 100 person years. The multivariate Cox regression analysis indicated higher NYHA class (HR 6.90, 95% CI 2.28-20.94, p = 0.001), telangiectasia (HR 4.18, 95% CI 1.25-13.92, p = 0.020), and enlarged LA diameter (HR 1.16, 95% CI 1.05-1.28, p = 0.005) as predictors of PH. Raynaud's phenomenon (HR 0.22, 95% CI 0.06-0.84, p = 0.026) and high oxygen saturation (HR 0.80, 95% CI 0.65-0.99, p = 0.047) were protective factors. The survival rate after PH diagnosis at 1, 3, and 5 years were 88.9%, 82.3%, and 48.0%, respectively. CONCLUSIONS: In this study cohort, the majority had early DcSSc, the incidence of PH was modest, and all cases developed concomitantly with ILD or left heart disease, resulting in poor survival. The presence of higher NYHA class, telangiectasia, and enlarged LA diameter was predictors of secondary PH. Further study regarding the treatment strategies for PH associated with ILD and left heart disease in SSc is needed. Key Points • In this cohort of early SSc in which the main subtype was DcSSc, the incidence of PH was modest and all PH was secondary PH associated with ILD and left heart disease, resulting in poor survival. • The presence of higher NYHA functional class, telangiectasia, and enlarged LA diameter was baseline predictors of developing secondary PH. • Effective treatment strategies for secondary PH due to ILD and cardiac involvement in SSc patients are urgently needed.

Worsening of esophageal dilatation is associated with increase in a high-resolution computed tomography (HRCT) score in early systemic sclerosis-associated interstitial lung disease (SSc-ILD).

BACKGROUND: Data regarding longitudinal association between changes (Δ: time2-time1) in the widest esophageal diameter (WED) and Δ HRCT score in early SSc-ILD patients is limited. We therefore investigated the association of ΔWED with Δ HRCT score and predictors of a worse Δ HRCT score in those patients. METHODS: We used an inception cohort of early SSc-ILD patients with availability for two HRCT records at enrollment and 1-year follow-up.The extent of ground glass, reticulation, bronchiectasis, and honeycombing was scored and then aggregated to produce a total HRCT score. The WED was measured at four levels and the maximum value was used. The Δ maximum WED, Δ mean WED, and Δ tHRCT score were analyzed. RESULTS: We recruited 75 early SSc-ILD patients and found a significant correlation of Δ tHRCT score with a Δ maximum WED (rho = 0.34, p < 0.01) and Δ mean WED (rho = 0.26, p < 0.05). There were 34 patients with a worsening Δ tHRCT (Δ > 0), 17 with stability (Δ = 0), and 24 with improvement (Δ < 0). Patients with a worsening ILD had a significantly shorter disease duration, lower prevalence of tendon friction rub, higher cumulative prednisolone dose, and larger ΔWED than those with stable and improved Δ tHRCT scores. Multivariate ordinal logistic regression identified a larger Δ mean WED (OR 1.21, 95% CI 1.03-1.42, p = 0.02) as a predictor of worsening HRCT score, while presence of tendon friction rub was associated with a lower risk (OR 0.18, 95% CI 0.04-0.77, p = 0.021). CONCLUSION: Our study cohort found that a worsening esophageal diameter was a predictor of progression of lung fibrosis determined by HRCT score in early SSc-ILD. A further study regarding esophageal dilation progression different in early versus longstanding SSc-ILD is needed. Key Points •In early SSc-ILD patients, we demonstrated that a worsening esophageal diameter was a predictor of progression of HRCT score at 1-year follow-up. •Further study regarding the association of worsening of the esophageal dilatation with the progression of ILD comparing between early versus late SSc-ILD is needed.

Rapid skin thickness progression rate is associated with high incidence rate of cardiopulmonary complications in patients with early diffuse cutaneous systemic sclerosis: inception cohort study.

OBJECTIVES: We aimed to investigate patients with early diffuse cutaneous systemic sclerosis (dcSSc) with regard to: 1. the association between skin thickness progression rate (STPR) at baseline visit and incidence rate of cardiopulmonary complications; 2. comparison of the mortality rate between patients with skin improvers and those with skin non-improvers. METHODS: An inception cohort of early dcSSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, Thailand, was selected. All patients were assessed for clinical manifestations, and modified Rodnan skin score (mRSS) and underwent echocardiography, and HRCT at study entry and then annually. RESULTS: One hundred and four dcSSc patients (57 of whom were females and 91 anti-topoisomerase I-positive) with a mean disease duration of 11.1±8.6 months were enrolled. Forty-two patients had rapid STPR [RPsp], 38 intermediate STPR [IMsp] and 24 slow STPR [SLsp]. At enrolment, the RPsp group had a significantly shorter disease duration, more prevalent anti-topoisomerase-I-positive, higher mRSS, more prevalent creatine kinase≥500 IU/L and higher NT-proBNP levels compared to the IMSp and SLsp groups. During a mean observation period of 4.5±2.0 years, the RPsp group had a significantly higher incidence rate of LVEF< 50% (6.06 vs. 0 per 100 person- years, p=<0.01) and interstitial lung disease (ILD) (69.69 vs. 34.66 per 100 person-years, p=0.012) than the SLsp group. Skin non-improvers had a signif- icantly higher mortality rate than skin improvers (28.6% vs. 5.8 %, p= 0.004). CONCLUSIONS: In this early dcSSc study cohort it was found that skin change determined by STPR at the baseline visit was a useful surrogate marker for cardiac and ILD complications. It was also found that skin improvers assessed 1-year later were a useful surrogate marker of mortality.

Contribution of HLA-B*51:01 and -A*26:01 to Behçet's disease and their clinical association in Thai patients.

AIMS: To investigate susceptible human leukocyte antigen (HLA) alleles and their associations with clinical features in Thai patients with Behçet's disease (BD). METHOD: Eighteen HLA-A and 36 HLA-B alleles were determined in 42 Thai BD patients and 99 healthy controls (HCs) by reverse line blot assay, and reconfirmed by MICRO SSP assay. RESULTS: The BD patients had significantly higher allele frequency (AF) of HLA-B*51 than the HCs (13.10% vs 5.05%, P = .025). The AF of HLA-A*26, -A*26:01 and -B*51:01 also was higher and almost reached statistical significance (5.59% vs 1.52%, P = .054, 5.95% vs 1.52%, P = .054 and 10.71% vs 4.04%, P = .051, respectively). However, the BD patients had significantly higher AF of either HLA-A*26:01 or -B*51:01 (16.67% vs 5.56%, P = .005), or -A*26:01 or -B*51X (19.05% vs 6.56%, P = .003). The AF of HLA-B*51:01 and -B*51X increased significantly in -A*26:01 non-carrier BD patients (12.16% vs 4.17%, P = .024 and 14.86% vs 5.21%, P = .019, respectively); and that of HLA-A*26:01 was significantly higher in -B*51X non-carrier BD patients (7.58% vs 1.67%, P = .034). HLA-B*51:01 associated significantly with the presence of posterior uveitis and visual impairment (18.18% vs 2.50%, P = .031 for both conditions). HLA-B*51:01 was not observed in BD patients with gastrointestinal involvement or arthritis. Furthermore, the AF of HLA-B*51:01 was significantly higher in HLA-A*26:01 non-carrier BD patients without arthritis (17.30% vs 0%, P = .050). CONCLUSION: HLA-B*51:01 was a susceptible allele for Thai BD patients, and associated with posterior uveitis and visual impairment. HLA-A*26:01 was another susceptible allele in HLA-B*51X non-carrier patients. The protective effect of HLA-B*51:01 on arthritis needs further investigation.

Comparison of clinical presentation and incidence of cardiopulmonary complications between male and female Thai patients with early systemic sclerosis: inception cohort study.

OBJECTIVES: To determine the prevalence of clinical manifestations and incidence rate of cardiopulmonary complications in a comparison between men and women with early SSc. METHODS: An inception cohort of early-SSc patients at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was used. All patients were assessed for clinical manifestations and underwent ECG, echocardiography, and HRCT at the study entry and then annually. RESULTS: One hundred and fifteen patients (46 male, 90 dcSSc) with a mean (SD) disease duration of 11.6 months (8.8) at cohort entry were enrolled during a mean (SD) observational period of 3.8 years (1.6). At enrollment, the male group had a higher prevalence of dcSSc subtype (91.3% vs. 69.5%, p = 0.006), hypo-hyperpigmentation (84.8% vs. 65.2%, p = 0.021), myositis (26.1% vs. 10.1%, p = 0.024), and right ventricular dysfunction [RVD] (8.7% vs. 0%, p = 0.024) compared with women. At the last visit, the male group had a higher cumulative prevalence of digital ulcers (47.8% vs. 27.5%, p = 0.026), telangiectasia (93.5% vs. 69.6%, p = 0.002), joint contracture (69.6% vs. 43.5%, p = 0.006), tendon friction rub (39.1% vs. 20.3%, p = 0.027), LVEF < 50% (21.7% vs. 8.7%, p = 0.048), and RVD (34.8% vs. 7.2%, p < 0.001). The male group had a significantly higher incidence rate of RVD (8.21 vs. 1.99 per 100 person-years, p = 0.006) and interstitial lung disease [ILD] (65.25 vs. 40.36 per 100 person-years, p = 0.022) compared to women. CONCLUSIONS: In this study cohort, it was found that men with SSc had more severe clinical manifestations and higher incidence rate of RVD and ILD compared to women. Increased awareness of cardiopulmonary complications in men even in early phase of SSc is crucial. KEY POINTS: • Male patients with SSc have more severe disease manifestations compared to women. • Even in the early phase of the disease, men were found to have higher incidence rates of right ventricular dysfunction and interstitial lung disease than women. • Increased awareness regarding cardiopulmonary complications in men with early SSc is crucial for effective management of these complications.

Clinical Manifestation and Incidence of Cardiopulmonary Complications in Early Systemic Sclerosis Patients with Different Antibody Profiles.

BACKGROUND: There has been no prior inception cohort study comparing clinical manifestations and incidence rate (IR) of cardiopulmonary involvement among early systemic sclerosis (SSc) patients by difference in autoantibody profiles. We compared the differences in the clinical presentation at study entry and cumulative organ complications at last visit, as well as the IR of cardiopulmonary complications between anti-topoisomerase I antibody-positive SSc patients (pATA), ATA-negative (nATA), and the positive anti-centromere antibody patients (pACA). METHODS: An inception cohort of early diagnosis SSc patients (disease duration ≤ 3 years) seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was studied. SSc patients who had follow-up duration as less than 1 year and those diagnosed with an overlap syndrome were excluded. All participants underwent electrocardiography (ECG), echocardiography, and high-resolution computed tomography (HRCT) at the study entry and then annually. RESULTS: A total of 114 patients (90 diffuse cutaneous SSc (dcSSc), 69 women) with mean (standard deviation, SD) disease duration of 11.7 (8.8) months at cohort entry and an observational period of 3.8 (1.6) years, were recruited. There were 89 patients (78.1%) with pATA, 18 (15.8%) with nATA, and 7 (6.1%) with pACA. At enrollment, both pATA and nATA groups had a higher prevalence of dcSSc subtype, and interstitial lung disease (ILD) when compared with the pACA group. At the last visit, the pATA group had a higher cumulative prevalence of digital ulcers, joint contracture and tendon friction rub than the other groups. Both the pATA and nATA groups had a significantly higher IR of ILD compared to the pACA group (54.9 and 57.8 vs. 6.3 per 100 person-years). During the study period, no suspected myositis, systolic pulmonary artery pressure (sPAP) ≥ 50 mm Hg or cardiac complications was observed in the pACA group. CONCLUSIONS: In our study cohort, the majority of which were dcSSc subtype with pATA, it was found that the presence of SSc-specific autoantibodies was associated with a distinctive clinical presentation and cumulative internal organ involvement, even in the early phase of the disease. Cardiopulmonary complications were rarely seen in the pACA group; whereas ILD complications were very common in both the pATA and nATA groups. A further study into the association of autoantibodies in nATA patients with ILD complications is needed.

Differences in clinical presentation and incidence of cardiopulmonary involvement in late-onset versus early-onset systemic sclerosis: inception cohort study.

INTRODUCTION: Data regarding the incidence rate (IR) of cardiopulmonary involvement in comparison between late-onset SSc and early-onset SSc are limited. OBJECTIVE: To compare the prevalence of clinical manifestations and the IR of cardiopulmonary involvement compared between the two subgroups. METHODS: An inception cohort of SSc patients seen at the Rheumatology Clinic, Maharaj Nakorn Chiang Mai Hospital, between January 2010 and June 2016, was used. All patients were assessed for clinical manifestations and underwent electrocardiograph, echocardiography and high-resolution computed tomography at the study entry and every 12 months thereafter. RESULT: One hundred and fifteen patients (69 female and 90 diffuse cutaneous SSc [dcSSc]) with a mean (SD) disease duration of 11.6 months (8.8) at cohort entry were enrolled during a mean (SD) observation period of 3.8 years (1.6). Patients were classified into two groups: age ≥ 50 years (late onset) and age < 50 years (early onset). The late-onset group included 78 patients (67.8%). At enrollment, the late-onset group had higher prevalence of digital pitting scars (60.3% vs. 35.1%, P = 0.012), dry eye symptoms (17.9% vs. 2.7%, P = 0.035), and hypertension (20.5% vs. 5.4%, P = 0.037) compared to the early-onset group. In the last visit, it was found that the late-onset group had higher cumulative prevalence of joint contracture (61.5% vs. 37.8%, P = 0.017) compared to the early-onset group. The late-onset group had no significant IR of left ventricular ejection fraction < 50% (3.04 vs. 4.45 per 100 person-years, P = 0.486), right ventricular dysfunction (5.17 vs. 2.73 per 100 person-years, P = 0.269), interstitial lung disease (49.45 vs. 42.03 per 100 person-years, P = 0.462), and systolic pulmonary arterial pressure ≥ 50 mmHg (2.57 vs. 1.07 per 100 person-years, P = 0.267) compared to the early-onset group. CONCLUSION: Our study cohort found that digital pitting scar, xerophthalmia, hypo-hyperpigmentation, joint contracture, and hypertension are more prevalent in late-onset SSc than early-onset SSc. However, no significant differences regarding the IR of cardiopulmonary involvement between the two subgroups, the majority of which were dcSSc, in the early phase of the disease.

Prevalence and predictors of hand involvement in Thai patients with systemic sclerosis.

AIM: Data regarding the clinical and radiographic hand involvement in Asian patients with systemic sclerosis (SSc) are limited. Thus, we determined the prevalence of clinical and radiographic hand involvement in Thai SSc patients, comparing diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc). We also determined the factors associated with arthritis, contracture of fingers and digital ulcers. METHOD: SSc patients seen at the Rheumatology Clinic, Chiang Mai University, Thailand, from December 2012 to June 2013 were consecutively invited to enroll in the study. After study entry, demographic data, clinical features and hand radiographs were evaluated. RESULT: We studied 110 SSc patients (73 dcSSc) with mean ± SD age of 53.2 ± 9.2 years and disease duration from non-Raynaud's phenomenon of 4.9 ± 4.8 years. The prevalence of arthritis, finger contractures and digital ulcers were 10 (9.1%), 47 (42.7%), and 14 (12.7%), respectively. DcSSc patients had significantly more of the following hand complications than lcSSc patients: digital pitting scar (53.4% vs. 27.0%, P = 0.008), digital ulcer (17.8% vs. 2.7%, P = 0.032), traumatic ulcer (27.4% vs. 0%, P < 0.001), acrolysis (45.2% vs. 18.9%, P = 0.007) and flexion contracture (60.3% vs. 8.1%, P < 0.001). Radiographic finger contractures were more prevalent in the dcSSc subset. In multivariate logistic regression analysis, a positive rheumatoid factor was associated with arthritis; dcSSc, arthritis and modified Rodnan skin score (MRSS) > 18 were associated with contracture of fingers. Furthermore, hand MRSS > 4 was associated with digital ulcers. CONCLUSION: Our results confirm that dcSSc patients had more severe clinical hand complications than lcSSc. However, radiographic findings were similar among subgroups, except that more finger contractures were seen in dcSSc. Finally, the presence of rheumatoid factor is associated with arthritis, and high MRSS is associated with finger contractures and digital ulcers.

Causes of death, survival and risk factors of mortality in Thai patients with early systemic sclerosis: inception cohort study.

Inception cohort study regarding the causes of death and risk factors for mortality in patients with early systemic sclerosis (SSc), especially diffuse SSc (dcSSc) has not been well elucidated. Therefore, the aim of our study was to determine the causes of death, survival rates, and risk factors for mortality in Thai patients with early SSc of whom the majority belonged to the dcSSc subset. We used an inception cohort of early-SSc patients seen between January 2010 and August 2014. All patients were evaluated for clinical and laboratory data at the study entry and then every 6 months. A total of 115 patients (68 female, 91 dcSSc) were enrolled. The mean ± SD age at onset, duration of disease, and duration of follow-up were 52.5 ± 8.5 years, 12.3 ± 9.2 months, and 27.5 ± 16.4 months, respectively. During the follow-up, 11(9.6%) SSc patients died. The mortality rate was 4.17 per 100 person-years (95% CI 2.31, 7.53). The leading cause of SSc-related death was dilated cardiomyopathy (27.2%). Infection was the most common cause of non-SSc-related death (18.2%). Survival rates at 1, 2, 3, and 4 years after the study entry were 93, 91, 88, and 88%, respectively. In the multivariate Cox regression analysis, ESR ≥ 40 mm/h [HR 8.65 (95% CI 1.66,45.17)], hemoglobin < 10 mg/dL [HR 4.57 (95% CI 1.14,18.34)], and mRSS [HR 1.09 (95% CI 1.03,1.15)] were independent risk factors for mortality. Our data suggest that dilated cardiomyopathy was the most common SSc-related cause of death in Thai patients with early SSc, of whom majority was dcSSc subset. Elevated ESR, anemia, and increased mRSS predicted poor outcome.

Risk factors and outcome of Thai patients with scleroderma renal crisis: a disease duration-matched case control study.

INTRODUCTION: Data regarding the prevalence, risk factors and outcome of scleroderma renal crisis (SRC) in Asian patients with systemic sclerosis (SSc) are limited. OBJECTIVE: To determine the prevalence, risk factors and outcomes of SRC in Thai SSc patients. METHOD: Medical records of all SSc patients seen at the Division of Rheumatology, Chiang Mai University, Thailand, from January 1990 to December 2015 were retrospectively reviewed. For each SRC case, a disease duration (±1 year) matched control (four SSc patient without SRC for each SRC patient) was identified. RESULT: Of 608 SSc patients seen during the study period, 19 SRC cases were identified, resulting in an SRC prevalence of 3.13%, with 76 matched controls. Of the 19 cases, mean ± SD age and median (interquartile range 1-3) disease duration was 56.2 ± 13.8 years and 5 (3-22) months, respectively. Seventeen patients (89.5%) had diffuse cutaneous SSc. Twelve patients (63.2%) had hypertensive renal crisis and seven (36.8%) had normotensive renal crisis. Multivariate conditional logistic regression analyses showed that digital gangrene (adjusted odd ratio [AOR] 31.41, 95% CI = 1.16-852.23, P = 0.041), current prednisolone dose ≥ 15 mg/day (AOR 31.22, 95% CI = 1.59-613.85, P = 0.024), serum albumin < 3 mg/dL (AOR 7.97, 95% CI = 1.49-42.56, P = 0.015), and cardiac involvement (AOR = 6.62, 95% CI = 1.08-40.63, P = 0.041) were independent risk factors for SRC. Fifteen SRC patients (78.9%) required dialysis and 10 (52.6%) died. CONCLUSION: SRC was an uncommon complication in Thai patients with SSc, but is associated with high mortality. Digital gangrene, current prednisolone dose ≥ 15 mg/day, serum albumin < 3 mg/dL and cardiac involvement were independent risk factors for SRC.

Correlation of delta high-resolution computed tomography (HRCT) score with delta clinical variables in early systemic sclerosis (SSc) patients.

BACKGROUND: The correlation of changes (delta: Δ) of high-resolution computed tomography (HRCT) score with the Δ of other clinical variables has not been well studied. The purpose of this study was to determine the correlation of Δ HRCT score with Δ percent predicted forced vital capacity (%pFVC), Δ modified Rodnan Skin Score (mRSS), Δ erythrocyte sedimentation rate (ESR), and Δ percent of oxygen saturation at room air (%SpO2) in patients with early systemic sclerosis (SSc). METHODS: We used an inception cohort of early-SSc patients seen at the Rheumatology Clinic, Chiang Mai University, Thailand, between January 2010 and June 2014. All patients underwent HRCT at study entry and every 12 months thereafter. Thirty-one SSc patients who underwent pulmonary function test (PFT) within 12 weeks of their corresponding HRCT at baseline and last visit were identified. The extent of ground glass (GG), lung fibrosis (Fib), bronchiectasis (B), and honeycombing (HC) was scored, and then aggregated to produce a total (t) HRCT score. RESULTS: Mean ± SD age and disease duration from non-Raynaud's phenomenon (NRP) to undergo HRCT at baseline were 52.2±8.8 years and 11.7±7.1 months, respectively. Seventeen (54.8%) patients were female and 20 (64.5%) were classified as dcSSc. The mean ± SD interval between the two HRCT tests was 16.0±7.2 months. The Δ HRCT scores [total fibrosis scores (t-Fib), total bronchiectasis scores (t-B), and total HRCT score (t-HRCT) scores] and Δ mRSS, but not Δ %pFVC, showed significant change over the observation period. We found significant correlation of Δ total honeycombing scores (t-HC) with Δ ESR (r=-0.44, P<0.05), and Δ t-Fib with Δ %SpO2 (r=-0.38, P<0.05). However, no significant correlation of any Δ HRCT scores with Δ %pFVC and Δ mRSS were observed. CONCLUSIONS: In this study, the changes in the HRCT scores were greater than %pFVC; this, along with their correlations with the changes in ESR and %SpO2, suggest that HRCT scores are a useful and sensitive method for monitoring disease progression in early SSc-related ILD (SSc-ILD).

Performance of the Existing Classification Criteria for Gout in Thai Patients Presenting With Acute Arthritis.

Currently, there are 5 existing classification criteria for gout: the Rome, New York, American Rheumatism Association (ARA), Mexico, and Netherlands criteria. This study was carried out to determine the performance of these classification criteria in Thai patients presenting with acute arthritis.All consecutive patients presenting with acute arthritis and being consulted at the Rheumatology Unit, Chiang Mai University Hospital from January 2013 to May 2015 were invited to join the study. Gout was defined by the presence of monosodium urate crystals in the synovial fluid or tissue examined by experienced rheumatologists. The 5 existing gout classification criteria were performed and evaluated in all of the patients, who were divided in subgroups of early disease (≤2 years), established disease (>2 years), and those without tophus.There were 136 gout and 97 nongout patients. Sensitivity and specificity across all criteria ranged from 75.7% to 97.1% and 68.0% to 84.5%, respectively. Overall, the Mexico criteria had the highest sensitivity (97.1%), and the ARA survey criteria the highest specificity (84.5%), whereas the Mexico criteria performed well in early disease with sensitivity and specificity of 97.1% and 81.7%, respectively. All 5 criteria showed high sensitivity (from 76.4% to 99.1%) but low specificity (from 30.8% to 65.4%) in established disease. In patients without tophus, the sensitivity and specificity ranged from 64.1% to 95.7% and 68.8% to 85.4%, respectively. The ARA survey criteria across all groups showed consistently high specificity for gout.The 5 existing classification criteria for gout had limited sensitivity and specificity in Thai patients presenting with acute arthritis. The ARA survey criteria are the most suitable for diagnosing gout in Thai people when crystal identification is not available.

The Correlation of Muscle Biopsy Scores with the Clinical Variables in Idiopathic Inflammatory Myopathies.

OBJECTIVES: To compare the muscle pathology findings among subgroups of idiopathic inflammatory myopathies (IIM) patients, and to determine the correlations of muscle biopsy scores with muscle power and creatine kinase (CK). METHODS: The medical records of IIM patients consisting of the demographic data, clinical parameters and laboratory conducted were retrospectively reviewed. Their initial muscle biopsies were reviewed, and four domains were scored: inflammation, vascular, muscle, and connective tissue. RESULTS: Ninety-five IIM patients (28 patients with idiopathic polymyositis (PM) 9 idiopathic dermatomyositis (DM), 5 DM associated with malignancy, and 53 PM/DM associated with connective tissue disease) with median (IQR: Q1, Q3) disease duration of 1.2 (0.5, 3.1) months were included. No significant differences in initial muscle pathology findings and muscle pathology score among the subgroups were found. Muscle degeneration and endomysial fibrosis scores were negatively correlated with muscle power (r=-0.23 and-0.24, respectively, p<0.05) and positively correlated with CK (r=0.27 and 0.39, respectively, p<0.01). No significant correlation was detected either inflammation or vasculitis scores with muscle power and CK levels. CONCLUSION: In this study, muscle biopsy cannot be used to differentiate among subgroups of IIM patients. In addition, we found only modest correlation of muscle biopsy scores with muscle power and CK. Further study is necessary to confirm our findings.

Incidence and predictors of interstitial lung disease (ILD) in Thai patients with early systemic sclerosis: Inception cohort study.

OBJECTIVES: To determine and compare the prevalence of interstitial lung disease (ILD), the severity of high-resolution computed tomography (HRCT) score and incidence rate (IR) of ILD between the two subsets of early-SSc (systemic sclerosis) patients. We also determined the factors associated with ILD. METHODS: We used an inception cohort of early-SSc patients seen between January 2010 and June 2014. All patients underwent HRCT at study entry and annually thereafter. RESULTS: One hundred and thirteen patients (66 females and 89 diffuse cutaneous SSc [dcSSc]) with a mean ± SD age of 53.4 ± 8.4 years and mean disease duration of 12.9 ± 10.3 months at cohort entry were enrolled. At enrollment, patients with dcSSc had a higher prevalence of ILD (78.7% vs. 45.8%, p = 0.002), and a higher total HRCT score (10.3 ± 9.5 vs. 4.4 ± 5.6, p = 0.001) compared with limited cutaneous SSc (lcSSc). DcSSc patients had a higher IR of ILD than lcSSc patients (58.8 vs.17.3 per 100 person-years, p < 0.001). Univariable analysis revealed that male gender, presence of anti-Scl 70 and absent anti-centromere antibody was significant predictors of ILD. In Cox-regression analysis, a positive anti-centromere [hazard ratio (HR) 0.09 95% confidence interval (95% CI 0.01-0.73)] was a protective factor. CONCLUSIONS: DcSSc patients had more severe HRCT scores and higher IR of ILD compared with lcSSc patients. Male gender, presence of anti-Scl 70, and absent anti-centromere antibody predicted the future development of ILD in early-SSc patients.

Deforming Arthropathy in Thai Patients With Systemic Lupus Erythematosus.

OBJECTIVE: The aim of this study was to determine the prevalence, spectrum, and clinical, radiological, and serologic findings as well as hand functions among Thai systemic lupus erythematosus (SLE) patients with deforming arthropathy. METHODS: All SLE patients attending the rheumatology clinic between January and December 2012 were interviewed, with their complete history and a physical examination being taken. Those with hand deformities were invited to join the study. RESULTS: Forty (8.7%) of 458 SLE patients had deforming arthropathy, with 13 (2.8%) of them having erosive arthritis (EA group) and 27 nonerosive arthropathy (NEA group) (8 [1.8%] with Jaccoud arthropathy [JA subgroup] and 19 [4.1%] with mild deforming arthropathy [MDA subgroup]). Three of 13 EA patients (0.7% of all SLE patients) had high titer of rheumatoid factor (RF) and anti-cyclic citrullinated peptide (CCP) antibodies that might represent true overlapping between rheumatoid arthritis and SLE. There were no statistically significant differences in autoantibodies, RF, or anti-CCP between the EA and NEA groups or the JA and MDA subgroups, except for discoid rash that was seen more commonly in the MDA subgroup. Rheumatoid factor and anti-CCP were not present in the JA subgroup. Hand joint destruction and deformities were seen more commonly in the EA group and JA subgroup. The hand grip and palmar pinch strength decreased moderately, with hand functions quite well preserved in all groups. CONCLUSIONS: Deforming arthropathy was not uncommon in Thai SLE patients, but true overlapping between rheumatoid arthritis and SLE was rare. Despite significant hand joint deformities and moderately decreased hand grip and palmar pinch strength, preservation of hand functions was generally apparent.

Effect of Antituberculous Drugs on Serum Uric Acid and Urine Uric Acid Excretion.

OBJECTIVES: The aim of this study was to determine the effect of antituberculous drugs on serum uric acid (SUA), urine uric acid (UUA) excretion, and renal function. METHODS: Patients with tuberculosis requiring a 6-month treatment course of antituberculous drugs (isoniazid, rifampicin, pyrazinamide, and ethambutol for 2 months, followed by isoniazid and rifampicin for a further 4 months) were included in this study. Serum uric acid, 24-hour UUA excretion, uric acid clearance (UACl), serum creatinine, and creatinine clearance were determined at baseline and at the end of the second week, second month, and fourth month. RESULTS: Sixteen of 50 patients completed the study. Their mean ± SD baseline SUA and UACl was 4.44 ± 1.72 mg/dL and 8.77 ± 7.03 mL/min per 1.73 m, respectively. At the second week, a significant increase in SUA (9.78 ± 3.21 mg/dL, P < 0.001) and significant decrease in UACl (3.50 ± 2.50 mL/min per 1.73 m, P = 0.001) were noted. These changes persisted through the second month, but returned to baseline value at the fourth month. Thirteen patients (81.25%) had hyperuricemia. The 24-hour UUA followed the same pattern as that of UACl but showed no statistical significance. There were no changes in serum creatinine or creatinine clearance. One patient had arthralgia, and another developed tuberculous arthritis. CONCLUSIONS: The hyperuricemic effect of pyrazinamide and ethambutol was due primarily to a decrease in UACl, which was reversible, and had no negative effect on the renal function. Arthralgia was uncommon and required no specific treatment.

Distribution of HLA-DR alleles among Thai patients with rheumatoid arthritis.

OBJECTIVE: This study was performed to investigate the association between the HLA-DR series and rheumatoid arthritis (RA) in a Thai population. METHODS: HLA-DR subtypes were determined in 100 Thai RA patients and 99 healthy controls (HC). HLA-DR typing was performed using INNO-LiPA HLA-DRB Decoder kits (Innogenetics) and reconfirmed using MICRO SSP HLA DNA Typing kits (One Lambda) for DRB1(∗)02 and (∗)04. RESULTS: When compared with the HC, the RA patients had higher allele frequency (AF) of DRB1(∗)04:05 (15.00% vs 7.07%, p=0.016, pc=NS, OR=2.319, 95%CI=1.189-4.522) and DRB1(∗)10:01 (3.00% vs 0%, p=0.030, pc=NS), respectively. The DRB1(∗)09:01 was slightly higher in the RA patients, without statistical significance. The AF of the shared epitope (SE) alleles (HLA-DRB1(∗)01:01, (∗)04:01, (∗)04:05 and (∗)10:01) was significantly higher in the RA patients (18.50% vs 7.58%, p=0.002, pc=0.046, OR=2.769, 95%CI=1.466-5.231, 99%CI=1.201-6.388). The AF of HLA-DRB4(∗)01 also increased significantly more in the RA patients (40.50% vs 25.76%, p=0.002, pc=0.002, OR=1.962, 95%CI=1.282-3.003, 99%CI=1.121-3.433). The HLA-DRB3(∗)03:01 was significantly lower in the RA patients (6.00% vs 14.14%, p=0.008, pc=0.023, OR=0.388, 95%CI=0.191-0.786, 99%CI=0.153-0.982). CONCLUSION: In the presence of SE, the DRB1(∗)04:05 and HLA-DRB4(∗)01 were associated with RA, and the DRB3(∗)03:01 would be a protective allele against RA in a Thai population.

The clinically quiescent phase in early-diagnosed SLE patients: inception cohort study.

OBJECTIVES: The aims of this study were to evaluate the incidence of clinical quiescence in early-diagnosed SLE patients and to determine factors associated with a prolonged clinically quiescent phase. METHODS: We used an inception cohort of SLE patients seen between May 2007 and June 2012. All patients were assessed for clinical quiescence [modified SLEDAI 2000 (mSLEDAI-2K) score = 0, no new features of lupus activity o increase in treatment] then evaluated for the occurrence of flare (mSLEDAI-2K increase ≥4 and increased disease activity in one or more organ systems or an increase in treatment). RESULTS: Ninety-five patients (88 females) with a mean age of 33.22 years (s.d. 13.24) and mean disease duration 2.79 months (s.d. 3.19) at cohort entry were enrolled during a mean observation period of 3.04 years (s.d. 1.38). Sixty-six patients (69.5%) reached clinical quiescence within 11.31 months (s.d. 1.10) of enrolment. Thirty-six patients (54.5%) had a disease flare during the observation period. The clinically quiescent phase was 28.2 months (s.d. 3.4). Cox regression analysis revealed that age ≥25 years at diagnosis [hazard ratio (HR) 2.57 (95% CI 1.23, 5.40)] and continued antimalarial drug treatment [HR 2.80 (95% CI 1.40, 5.58)] were associated with a longer clinically quiescent phase. CONCLUSION: Most early-diagnosed SLE patients could have a good prognosis. Age at diagnosis ≥25 years or continued treatment with antimalarial drugs after reaching clinical quiescence may result in a longer clinically quiescent phase. More studies are needed to elucidate the mechanism of action for these protective effects.