Risk of flare and damage accrual after tapering glucocorticoids in modified serologically active clinically quiescent patients with systemic lupus erythematosus: a multinational observational cohort study.

OBJECTIVES: To assess the risk of flare and damage accrual after tapering glucocorticoids (GCs) in modified serologically active clinically quiescent (mSACQ) patients with systemic lupus erythematosus (SLE). METHODS: Data from a 12-country longitudinal SLE cohort, collected prospectively between 2013 and 2020, were analysed. SLE patients with mSACQ defined as the state with serological activity (increased anti-dsDNA and/or hypocomplementemia) but without clinical activity, treated with ≤7.5 mg/day of prednisolone-equivalent GCs and not-considering duration, were studied. The risk of subsequent flare or damage accrual per 1 mg decrease of prednisolone was assessed using Cox proportional hazard models while adjusting for confounders. Observation periods were 2 years and censored if each event occurred. RESULTS: Data from 1850 mSACQ patients were analysed: 742, 271 and 180 patients experienced overall flare, severe flare and damage accrual, respectively. Tapering GCs by 1 mg/day of prednisolone was not associated with increased risk of overall or severe flare: adjusted HRs 1.02 (95% CI, 0.99 to 1.05) and 0.98 (95% CI, 0.96 to 1.004), respectively. Antimalarial use was associated with decreased flare risk. Tapering GCs was associated with decreased risk of damage accrual (adjusted HR 0.96, 95% CI, 0.93 to 0.99) in the patients whose initial prednisolone dosages were >5 mg/day. CONCLUSIONS: In mSACQ patients, tapering GCs was not associated with increased flare risk. Antimalarial use was associated with decreased flare risk. Tapering GCs protected mSACQ patients treated with >5 mg/day of prednisolone against damage accrual. These findings suggest that cautious GC tapering is feasible and can reduce GC use in mSACQ patients.

SMART-SLE: serology monitoring and repeat testing in systemic lupus erythematosus-an analysis of anti-double-stranded DNA monitoring.

OBJECTIVE: Disease activity monitoring in SLE includes serial measurement of anti-double stranded-DNA (dsDNA) antibodies, but in patients who are persistently anti-dsDNA positive, the utility of repeated measurement is unclear. We investigated the usefulness of serial anti-dsDNA testing in predicting flare in SLE patients who are persistently anti-dsDNA positive. METHODS: Data were analysed from patients in a multinational longitudinal cohort with known anti-dsDNA results from 2013 to 2021. Patients were categorized based on their anti-dsDNA results as persistently negative, fluctuating or persistently positive. Cox regression models were used to examine longitudinal associations of anti-dsDNA results with flare. RESULTS: Data from 37 582 visits of 3484 patients were analysed. Of the patients 1029 (29.5%) had persistently positive anti-dsDNA and 1195 (34.3%) had fluctuating results. Anti-dsDNA expressed as a ratio to the normal cut-off was associated with the risk of subsequent flare, including in the persistently positive cohort (adjusted hazard ratio [HR] 1.56; 95% CI: 1.30, 1.87; P < 0.001) and fluctuating cohort (adjusted HR 1.46; 95% CI: 1.28, 1.66), both for a ratio >3. Both increases and decreases in anti-dsDNA more than 2-fold compared with the previous visit were associated with increased risk of flare in the fluctuating cohort (adjusted HR 1.33; 95% CI: 1.08, 1.65; P = 0.008) and the persistently positive cohort (adjusted HR 1.36; 95% CI: 1.08, 1.71; P = 0.009). CONCLUSION: Absolute value and change in anti-dsDNA titres predict flares, including in persistently anti-dsDNA positive patients. This indicates that repeat monitoring of dsDNA has value in routine testing.

Comparison of Attainment and Protective Effects of Lupus Low Disease Activity State In Patients With Newly Diagnosed Versus Established Systemic Lupus Erythematosus.

OBJECTIVE: To assess whether Lupus Low Disease Activity State (LLDAS) attainment is associated with favorable outcomes in patients with recent onset systemic lupus erythematosus (SLE). METHODS: Data from a 13-country longitudinal SLE cohort were collected prospectively between 2013 and 2020. An inception cohort was defined based on disease duration < 1 year at enrollment. Patient characteristics between inception and noninception cohorts were compared. Survival analyses were performed to examine the association between LLDAS attainment and damage accrual and flare. RESULTS: Of the total 4106 patients, 680 (16.6%) were recruited within 1 year of SLE diagnosis (inception cohort). Compared to the noninception cohort, inception cohort patients were significantly younger, had higher disease activity, and used more glucocorticoids, but had less organ damage at enrollment. Significantly fewer inception cohort patients were in LLDAS at enrollment than the noninception cohort (29.6% vs 52.3%, P < 0.001), but three-quarters of both groups achieved LLDAS at least once during follow-up. Limiting analysis only to patients not in LLDAS at enrollment, inception cohort patients were 60% more likely to attain LLDAS (hazard ratio 1.37, 95% CI 1.16-1.61, P < 0.001) than noninception cohort patients and attained LLDAS significantly faster. LLDAS attainment was significantly protective against flare in both the inception and noninception cohorts. A total of 88 (13.6%) inception cohort patients accrued organ damage during a median 2.2 years of follow-up. CONCLUSION: LLDAS attainment is protective from flare in recent onset SLE. Significant protection from damage accrual was not observed because of low rates of damage accrual in the first years after SLE diagnosis. (ClinicalTrials.gov: NCT03138941).

Prevalence and characteristics of inflammatory rheumatic diseases in patients with thalassemia.

Reports of inflammatory rheumatic diseases (IRD) in thalassemia are limited. This study aimed to determine the prevalence and clinical characteristics of IRD in patients with thalassemia disease. Consecutive adult patients with thalassemia disease, confirmed by hemoglobin typing, attending the Hematology Clinic between June 2019 and May 2021 were invited to join this study. All of them had their history taken and a physical examination for IRD. Those with IRD had their medical records reviewed. Sixty-three patients (transfusion-dependent in 50) were included in this study. There was α-, ß-, and co-inheritance of α- and ß-thalassemia in 22.22%, 73.02%, and 4.76% of the patients, respectively, with ß-thalassemia/Hb E disease in 53.97%. Twenty-three patients had IRD (rheumatoid arthritis in 9, gout in 6, systemic lupus erythematosus in 3, spondyloarthropathy in 2, and one patient each with dermatomyositis, overlap syndrome, and unclassified polyarthralgia). Clinical manifestations and laboratory findings were similar to IRD patients in general. In 40 patients without IRD, direct and indirect Coombs tests and antinuclear antibody (ANA) were positive in 51.72%, 27.59%, and 10.26%, respectively. When comparing among these 40 patients, between those with non-transfusion-dependent thalassemia (n = 10) and those with transfusion-dependent thalassemia (n = 30), the latter had non-significantly more positive direct Coombs (60.87% vs. 16.67%), indirect Coombs (30.43% vs. 16.67%), and ANA tests (13.33% vs. 0%). The prevalence of IRD in patients with thalassemia disease was rather high. Positive direct Coombs test and ANA were common in transfusion-dependent patients.

Acute parkinsonism in patients with systemic lupus erythematosus: a case report and review of the literature.

Purpose of the study: Parkinsonism in patients with systemic lupus erythematosus (SLE) is rare. This study reported a case of parkinsonism in SLE and reviewed the clinical features and outcomes of parkinsonism in SLE patients.Methods: English language literature of parkinsonism in SLE patients was reviewed.Results: There were 28 patients (19 adults and 9 children) with SLE and parkinsonism. Twenty-three patients were female. Of 26 patients whose disease duration was available parkinsonism occurred at SLE diagnosis and after SLE diagnosis in 6 and 20 patients, respectively. Twenty-five patients had active SLE. Hematologic, mucocutaneous and musculoskeletal systems were the 3 most common organs involved in SLE during parkinsonism onset. Rigidity, bradykinesia and resting tremor were the 3 most common parkinsonian symptoms. Compared with adults, child cases had significantly more psychosis (4 in 9 vs. 1 in 19, p = .026), seizures or psychosis (6 in 9 vs. 2 in 19, p = .005) and mutism (6 in 9 vs. none, p < .001). Brain magnetic resonance imaging (MRI) was abnormal in 13 of 24 patients. Eight of nine patients had abnormal single-photon emission computed tomography (SPECT) and 5 and 3 showed hypoperfusion and hyperperfusion, respectively. The outcomes were resolution, partial response and persistent symptoms in 17, 7 and 4 patients, respectively. The outcome was no different whether or not dopamine therapy was included to corticosteroids and/or immunosuppressive drugs.Conclusions: Parkinsonism in SLE usually occurs during active SLE disease. Good response to corticosteroid and/or immunosuppressive drugs supports the immunologic mechanism in the pathogenesis.

Disease Activity and Rate and Severity of Flares During Peripartum Period in Thai Patients With Systemic Lupus Erythematosus: An Age at Diagnosis and Disease Duration Matched Controlled Study.

BACKGROUND/OBJECTIVE: The aim of this study was to compare disease activity and rate and severity of flares between pregnant and nonpregnant systemic lupus erythematosus (SLE) patients. METHODS: Medical records of pregnant SLE patients seen between January 1993 and June 2017 were reviewed. Nonpregnant SLE controls were matched by age at diagnosis and disease duration before pregnancy. Systemic lupus erythematosus disease activity and flares were determined by the cSLEDAI (clinical Systemic Lupus Erythematosus Disease Activity Index) and Safety of Estrogens in Lupus Erythematosus National Assessment-SLEDAI Flare Index, respectively. Disease activity was measured from 6 months before conception (-6 months) until the postpartum period. The repeated measures mixed model, Cox regression, and cumulative hazard plots were used for statistical analysis. RESULTS: Ninety pregnancies occurred in 77 patients. The cSLEDAI scores from -6 months to the postpartum period were comparable between the pregnancy and control group, but slightly yet significantly higher in the controls at conception (mean ± SEM, 3.57 ± 0.45 vs 1.90 ± 0.36; p = 0.019). When compared with the controls, during the pregnancy and postpartum period, the pregnancy group did not have significantly higher incidence of flare (41.11% vs 28.89%, p = 0.086 and 7.78% vs 11.11%, p = 0.445, respectively) or flare category (severe flare) (75.68% vs 53.85%, p = 0.070 and 85.71% vs 70.00%, p = 0.603, respectively). The flare incidence rate (95% confidence interval)/100 patient-months in the pregnancy and control group was 6.75 (4.89-9.32) and 4.34 (2.96-6.38), respectively, giving the adjusted hazards for flare (95% confidence interval) of 1.54 (0.91-2.61) (p = 0.110). CONCLUSIONS: There was no overall significant increase in SLE disease activity, flare incidence, and flare severity in pregnant SLE patients when compared with their properly matched nonpregnant SLE controls.

Independent associations of lymphopenia and neutropenia in patients with systemic lupus erythematosus: a longitudinal, multinational study.

OBJECTIVE: The prevalence and associations of leucopenia in SLE remain incompletely understood. We evaluated associations of disease activity and medication use with leucopenia (lymphopenia and neutropenia) in a multinational, prospectively followed SLE cohort. METHODS: Data from the Asia Pacific Lupus Collaboration cohort, in which disease activity and medications were prospectively captured from 2013 to 2018, were used. Predictors of lymphopenia (lymphocyte count <0.8 × 109/l) and neutropenia (neutrophil count <1.5 × 109/l) were examined using multiple failure, time-dependent survival analyses. RESULTS: Data from 2330 patients and 18 287 visits were analysed. One thousand and eighteen patients (43.7%) had at least one episode of leucopenia; 867 patients (37.2%) had lymphopenia, observed in 3065 (16.8%) visits, and 292 (12.5%) patients had neutropenia, in 622 (3.4%) visits. After multivariable analyses, lymphopenia was associated with overall disease activity, ESR, serology, prednisolone, AZA, MTX, tacrolimus, CYC and rituximab use. MTX and ciclosporin were negatively associated with neutropenia. Lupus low disease activity state was negatively associated with both lymphopenia and neutropenia. CONCLUSION: Both lymphopenia and neutropenia were common in SLE patients but were differentially associated with disease and treatment variables. Lymphopenia and neutropenia should be considered independently in studies in SLE.

Which factors predict discordance between a patient and physician on a gout flare?

OBJECTIVE: To investigate the factors associated with discordance between patient and physician on the presence of a gout flare. METHODS: Patients' self-reports of current gout flares were assessed with the question, 'Are you having a gout flare today?' which was then compared with a concurrent, blinded, physician's assessment. Based on agreement or disagreement with physicians on the presence of a gout flare, flares were divided into concordant and discordant groups, respectively. Within the discordant group, two subgroups-patient-reported flare but the physician disagreed and physician-reported flare but the patient disagreed-were identified. The factors associated with discordance were analysed with multivariable logistic regression analysis. RESULTS: Of 268 gout flares, 81 (30.2%) flares were discordant, with either patient or physician disagreeing on the presence of a flare. Of the discordant flares, in 57 (70.4%) the patient reported a flare but the physician disagreed. In multivariable logistic regression analysis adjusted for demographics, disagreement among patients and physicians on the presence of a gout flare was associated with lower pain scores at rest [odds ratio (OR) for each point increase on 0-10 point pain scale 0.81 (95% Wald CI 0.73, 0.90), P < 0.0001] and less presence of joint swelling [OR 0.24 (95% CI 0.10, 0.61), P = 0.003] or joint warmth [OR 0.39 (95% CI 0.20, 0.75), P = 0.005]. CONCLUSION: Although patients and physicians generally agree about the presence of gout flare, discordance may occur in the setting of low pain scores and in the absence of swollen or warm joints.

Associations between physicians' global assessment of disease activity and patient-reported outcomes in patients with systemic lupus erythematosus: A longitudinal study.

OBJECTIVE: To determine longitudinal associations between Physician Global Assessment (PGA) and patient-reported outcomes (PROs) in patients with systemic lupus erythematosus (SLE). METHODS: Patients attending a rheumatology clinic between 2013 and 2017 completed specific (SLEQOL) and generic (SF36) health-related quality of life (HRQoL) surveys and rated their global rating of change (GRC) at each visit. PGA, SLEDAI-2K and SLE Flare Index (SFI) were also captured on all visits. Generalised estimating equations (GEE) methods were used to examine longitudinal associations of PGA with PROs and clinical indicators. RESULTS: 337 patients were followed for a median [IQR] of 3.2 [1.6, 3.4] years (2,059 visits). High PGA (>1) was strongly associated with high SLEDAI-2K scores, the presence of flares and poor PROs. Odd ratios (OR) [95% CI] of PGA > 1 in patients with SLEDAI-2K >4 & <10 and SLEDAI-2K ≥10, compared to SLEDAI-2K ≤ 4, were 3.46 [2.36, 5.08], p < 0.001 and 6.39 [4.30, 9.49], p < 0.001, respectively. OR [95% CI] of PGA > 1 in patients with mild-to-moderate or severe flares were 2.09 [1.62, 2.71], p < 0.001 and 4.42 [3.21, 6.07], p < 0.001, respectively. Mental components of both SLEQOL (mood, self-image) and SF36 (MCS) surveys demonstrated significant associations with high PGA. After adjusting for SLEDAI-2K, one-point increase in PGA was associated with reductions in SLEQOL total score and SF36-MCS by 2.33 (regression coefficient (RC) [95% CI] = -2.33 [-3.77, -0.88], p = 0.002), and 4.16 (RC [95% CI] = -4.16 [-5.19, -3.13], p < 0.001) points, respectively. Associations of some physical components (SLEQOL-symptoms, and SF36-PCS) with PGA attenuated when adjusted for SLEDAI-2K. Patients who rated low scores of GRC, which indicate health deterioration, were twice as likely to have PGA > 1 (OR [95%CI] 1.99 [1.25, 3.16], p = 0.004). CONCLUSION: High PGA was strongly associated with poor mental health, high disease activity and flares. This study confirms the value of PGA as an efficient assessment tool for SLE.

Effect of caffeinated and decaffeinated coffee on serum uric acid and uric acid clearance, a randomised within-subject experimental study.

OBJECTIVES: The effect of coffee on serum uric acid (SUA) has shown conflicting results. This study was to determine the effects of caffeinated coffee (CC) and decaffeinated coffee (DC) on SUA, serum xanthine oxidase activity (sXOA) and urine uric acid clearance (UAC). METHODS: This was a prospective randomised within-subject experimental study design of 51 healthy male participants. Each study period consisted of 3 periods, including a control, an intervention, and washout period for 1, 3 and 1 week, respectively. During the intervention period, the participants received 2, 4 or 6 gram/day of coffee, either CC or DC. RESULTS: For DC groups, SUA significantly decreased by 6.5 (±1.1) mg/dL to 6.2 (±1.1) mg/dL during the intervention period (p=0.014). sXOA significantly increased by 0.05 (±0.07) nmol/min/mL to 0.20 (±0.38) nmol/min/mL during the intervention period (p=0.010) of CC. For UAC, there was no significant change with CC or DC. In hyperuricaemic participants, SUA significantly decreased by 7.7 (±0.7) mg/dL to 7.2 (±0.7) mg/dL during the intervention period (p=0.028) of DC. For non-hyperuricaemic, CC significantly increased SUA by 5.9 (±0.7) mg/dL to 6.2 (±0.9) mg/dL during the intervention period (p=0.008) and significantly decreased SUA to 6.0 (±0.8) mg/dL (p=0.049) during the withdrawal period. A significant increase of sXOA according with SUA in CC groups from 0.05 (±0.07) nmol/min/mL to 0.25 (±0.44) nmol/min/mL during the intervention period (p=0.040) was presented in non-hyperuricaemic participants. CONCLUSIONS: DC had a significant decrease of SUA during the intervention period. However, in non-HUS participants, SUA significantly increased in CC.

Hyperuricemia, urine uric excretion, and associated complications in thalassemia patients.

Thalassemia patients have a high cell turnover rate due to chronic hemolysis and ineffective erythropoiesis; therefore, hyperuricemia is anticipated. This study aimed to identify the prevalence of hyperuricemia, gout and nephrolithiasis, conditions associated with serum uric acid (SUA), and urine uric acid excretion (UUA) in thalassemia patients. This was a cross-sectional study in patients aged 15 years or older at Chiang Mai University Hospital. All patients had blood and 24-h urine collection test. We enrolled 112 thalassemia patients in which 67.0% were female, 64.3% had beta thalassemia/Hb E, 76.8% were transfusion dependent, and 59.8% were post splenectomy. The median age was 29 (16-58) years. Mean SUA was 6.7 ± 2.0 mg/dl and hyperuricemia (SUA > 6.8 mg/dl) was found in 47 cases (45.2%). Intact spleen (ORs 4.3, 95%CI 1.55-12.50, p = 0.01) and lower FEuric (ORs 2.08, 95%CI 1.35-3.33, p < 0.01) were associated with hyperuricemia significantly. Seven (6.3%) had gouty arthritis and nine (8%) had microscopic hematuria, one case being confirmed nephrolithiasis. The mean UUA excretion was 981.3 ± 335.0 mg/day and UUA hyperexcretion (> 700 mg/24 h) was found in 83.3%. UUA hyperexcretion patients had renal hyperfiltration 46%, glomerular dysfunction 84%, and tubular dysfunction 7.7%. From our study, hyperuricemia was found in approximately 40% of thalassemia patients but gouty arthritis occurred only in few patients (6%). This may be explained by urinary uric hyperexcretion which is found in over 80%. The significant risk factors for hyperuricemia were intact spleen and lower fraction excretion of uric acid.

Effectiveness and Drug Survival of Anti-Tumor Necrosis Factor α Therapies in Patients With Spondyloarthritis: Analysis From the Thai Rheumatic Disease Prior Authorization Registry.

OBJECTIVE: This study aimed to evaluate the long-term effectiveness and safety of the first anti-tumor necrosis factor α therapy (TNFi) and to identify the associated factors of drug discontinuation in patients with spondyloarthritis. METHODS: This was a medical records review study. Patients with spondyloarthritis who were prescribed the first TNFi between December 2009 and October 2014 in the Rheumatic Disease Prior Authorization registry were enrolled. Baseline clinical data were retrieved. The Cox proportional hazards model was used to identify factors associated with discontinuation of drugs. RESULTS: Among 138 patients, 97 had ankylosing spondylitis (AS), and 41 had psoriatic arthritis (PsA). The effectiveness of TNFi in AS and PsA was 55% to 59% at 4 months and 75% to 96% at 3 years, as measured by a 50% decrease in the Bath Ankylosing Spondylitis Disease Activity Index from baseline. For PsA with peripheral arthritis, improvement of the joint count by 50% was observed in 61.8% of patients at 4 months and 100% at 3 years. Survival from TNFi was 63% for AS and 56% for PsA at 3 years. For AS, the factors associated with good response leading to discontinuation of TNFi were baseline patient global assessment 3 to 6/10 (hazard ratio [HR], 6.3) and the use of leflunomide (HR, 6.0) and infliximab (HR, 4.8). A good response (38.5%) was the most common cause of discontinuation of the first TNFi, followed by toxicity (28.2%), nonadherence (20.5%), and lack of effectiveness (12.8%). CONCLUSIONS: Ankylosing spondylitis and PsA responded well to TNFi during the 3-year follow-up. The retention rate was approximately 60% for AS and PsA. A good response to the first TNFi was the most common reason for discontinuation.

Performance of the 2015 American College of Rheumatology/European League Against Rheumatism gout classification criteria in Thai patients.

To evaluate the sensitivity and specificity of the 2015 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) gout classification criteria in Thai patients presenting with acute arthritis in a real-life setting. Data were analyzed on consecutive patients presenting with arthritis of less than 2 weeks duration. Sensitivity and specificity were calculated by using the presence of monosodium urate (MSU) crystals in the synovial fluid or tissue aspirate as gold standard for gout diagnosis. Subgroup analysis was performed in patients with early disease (≤2 years), established disease (>2 years), and those without tophus. Additional analysis also was performed in non-tophaceous gout patients, and patients with acute calcium pyrophosphate dihydrate crystal arthritis were used as controls. One hundred and nine gout and 74 non-gout patients participated in this study. Full ACR/EULAR classification criteria had sensitivity and specificity of 90.2 and 90.0%, respectively; and 90.2 and 85.0%, respectively, when synovial fluid microscopy was excluded. Clinical-only criteria yielded sensitivity and specificity of 79.8 and 87.8%, respectively. The criteria performed well among patients with early and non-tophaceous disease, but had lower specificity in patients with established disease. The variation of serum uric acid level was a major limitation of the classification criteria. The ACR/EULAR classification criteria had high sensitivity and specificity in Thai patients presenting with acute arthritis, even when clinical criteria alone were used.

Value of ultrasonography in the diagnosis of gout in patients presenting with acute arthritis.

OBJECTIVE: To evaluate the value of ultrasonographic features of crystal deposition for diagnosing gout in patients presenting with acute arthritis. MATERIALS AND METHODS: Ultrasound scanning of the most inflamed joint was performed on 89 consecutively enrolled patients with acute arthritis. Two radiologists independently reviewed the ultrasound images, and a consensus was achieved with a third radiologist when the interpretations of four key ultrasound features of gout differed. Arthrocentesis and crystal analysis using compensated polarized light microscopy of aspirates are considered the gold standards for gout diagnosis. RESULTS: Fifty-three (60%) patients had gout, whereas the remaining 36 (40%) had non-gout arthritis. The mean serum uric acid level was 7.1 mg/dl in patients with gout and 4.7 mg/dl in patients with non-gout arthritis. Three US features differed significantly (p < 0.001) between patients with gout and non-gout arthritis: the double contour sign (42 vs. 8%, respectively), intra-articular aggregates (58 vs. 8%), and tophi (40 vs. 0%). No statistically significant differences in detecting intra-tendinous aggregates (32 vs. 17%, p = 0.14) were observed. The sensitivity and specificity of the double contour sign were 42 and 92%, respectively; those of the intra-articular aggregates were 58 and 92%; and those of tophi were 40 and 100%. The positive predictive values for these three features ranged from 88 to 100%, whereas the negative predictive values ranged from 52 to 60%. CONCLUSIONS: When the prevalence is high, these three ultrasound features may be a useful adjunct in the diagnosis of acute gout, particularly when specialized microscopic techniques are not available.

Definition and initial validation of a Lupus Low Disease Activity State (LLDAS).

AIMS: Treating to low disease activity is routine in rheumatoid arthritis, but no comparable goal has been defined for systemic lupus erythematosus (SLE). We sought to define and validate a Lupus Low Disease Activity State (LLDAS). METHODS: A consensus definition of LLDAS was generated using Delphi and nominal group techniques. Criterion validity was determined by measuring the ability of LLDAS attainment, in a single-centre SLE cohort, to predict non-accrual of irreversible organ damage, measured using the Systemic Lupus International Collaborating Clinics Damage Index (SDI). RESULTS: Consensus methodology led to the following definition of LLDAS: (1) SLE Disease Activity Index (SLEDAI)-2K ≤4, with no activity in major organ systems (renal, central nervous system (CNS), cardiopulmonary, vasculitis, fever) and no haemolytic anaemia or gastrointestinal activity; (2) no new lupus disease activity compared with the previous assessment; (3) a Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLEDAI physician global assessment (scale 0-3) ≤1; (4) a current prednisolone (or equivalent) dose ≤7.5 mg daily; and (5) well tolerated standard maintenance doses of immunosuppressive drugs and approved biological agents. Achievement of LLDAS was determined in 191 patients followed for a mean of 3.9 years. Patients who spent greater than 50% of their observed time in LLDAS had significantly reduced organ damage accrual compared with patients who spent less than 50% of their time in LLDAS (p=0.0007) and were significantly less likely to have an increase in SDI of ≥1 (relative risk 0.47, 95% CI 0.28 to 0.79, p=0.005). CONCLUSIONS: A definition of LLDAS has been generated, and preliminary validation demonstrates its attainment to be associated with improved outcomes in SLE.

Performance of classification criteria for gout in early and established disease.

OBJECTIVES: To compare the sensitivity and specificity of different classification criteria for gout in early and established disease. METHODS: This was a cross-sectional study of consecutive rheumatology clinic patients with joint swelling in which gout was defined by presence or absence of monosodium urate crystals as observed by a certified examiner at presentation. Early disease was defined as patient-reported onset of symptoms of 2 years or less. RESULTS: Data from 983 patients were collected and gout was present in 509 (52%). Early disease was present in 144 gout cases and 228 non-cases. Sensitivity across criteria was better in established disease (95.3% vs 84.1%, p<0.001) and specificity was better in early disease (79.9% vs 52.5%, p<0.001). The overall best performing clinical criteria were the Rome criteria with sensitivity/specificity in early and established disease of 60.3%/84.4% and 86.4%/63.6%. Criteria not requiring synovial fluid analysis had sensitivity and specificity of less than 80% in early and established disease. CONCLUSIONS: Existing classification criteria for gout have sensitivity of over 80% in early and established disease but currently available criteria that do not require synovial fluid analysis have inadequate specificity especially later in the disease. Classification criteria for gout with better specificity are required, although the findings should be cautiously applied to non-rheumatology clinic populations.

Acute bone infarction: a rare complication in thalassemia.

Acute bone infarction is a well-described complication in sickle cell hemoglobinopathy but it is rarely reported in patients with thalassemia. This report describes an 18-year-old man with homozygous ß-thalassemia presenting with a fever and severe acute bilateral ankle pain. The acute onset of severe pain and fever were clinical mimics of infectious arthritis and osteomyelitis. Magnetic resonance imaging revealed acute bone infarction in the meta-diaphysis of bilateral tibias presenting as central unenhanced devitalized bone with T1-high signal intensity fluid in the subperiosteum and soft tissue. Characteristic imaging features are discussed, emphasizing the benefit of fat suppression pre-and post-intravenous gadolinium T1-weighted images. The etiologies of bone infarction in thalassemia are reviewed.

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.

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.

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.