Publications by authors named "Andrea Hsiu Ling Low"

14 Publications

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Recommendations for COVID-19 vaccination in people with rheumatic disease: Developed by the Singapore Chapter of Rheumatologists.

Int J Rheum Dis 2021 Jun 10;24(6):746-757. Epub 2021 May 10.

Division of Rheumatology, Department of Medicine, National University Hospital, Singapore, Singapore.

Aim: People with rheumatic diseases (PRD) remain vulnerable in the era of the COVID-19 pandemic. We formulated recommendations to meet the urgent need for a consensus for vaccination against SARS-CoV-2 in PRD.

Methods: Systematic literature reviews were performed to evaluate: (a) outcomes in PRD with COVID-19; (b) efficacy, immunogenicity and safety of COVID-19 vaccination; and (c) published guidelines/recommendations for non-live, non-COVID-19 vaccinations in PRD. Recommendations were formulated based on the evidence and expert opinion according to the Grading of Recommendations Assessment, Development and Evaluation methodology.

Results: The consensus comprises 2 overarching principles and 7 recommendations. Vaccination against SARS-CoV-2 in PRD should be aligned with prevailing national policy and should be individualized through shared decision between the healthcare provider and patient. We strongly recommend that eligible PRD and household contacts be vaccinated against SARS-CoV-2. We conditionally recommended that the COVID-19 vaccine be administered during quiescent disease if possible. Immunomodulatory drugs, other than rituximab, can be continued alongside vaccination. We conditionally recommend that the COVID-19 vaccine be administered prior to commencing rituximab if possible. For patients on rituximab, the vaccine should be administered a minimum of 6 months after the last dose and/or 4 weeks prior to the next dose of rituximab. Post-vaccination antibody titers against SARS-CoV-2 need not be measured. Any of the approved COVID-19 vaccines may be used, with no particular preference.

Conclusion: These recommendations provide guidance for COVID-19 vaccination in PRD. Most recommendations in this consensus are conditional, reflecting a lack of evidence or low-level evidence.
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http://dx.doi.org/10.1111/1756-185X.14107DOI Listing
June 2021

Microbiome and osteoarthritis: New insights from animal and human studies.

Int J Rheum Dis 2021 May 7. Epub 2021 May 7.

Department of Rheumatology & Immunology, Singapore General Hospital, Singapore City, Singapore.

Osteoarthritis (OA) is a common cause of disability, especially among the elderly. With an ageing and increasingly obese population, OA will become more prevalent. Obesity and metabolic syndrome are risk factors for OA and have been implicated in its pathogenesis. The gut microbiome may shed light on this possible common pathogenesis. Recent animal and human studies have gained important insights into the relationship between OA, obesity, and the gut microbiome. Animal studies have demonstrated links between obesity and increased severity of OA and altered gut microbial DNA profile. Use of prebiotics and probiotics in animal trials provides proof-of-concept that interventional options to the gut microbiome can modulate the progression of OA favorably. Current evidence in human studies is limited. Shifts in gut microbial profile and reduced gut microbial diversity have been associated with people with OA, as well as blood and synovial fluid lipopolysaccharide endotoxemia. Linkages between microbiome dysbiosis and host responses may help in the understanding of OA pathogenesis and the discovery of therapeutic targets. This narrative review provides a summary of up-to-date animal and human studies on the gut microbiome and its link with OA.
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http://dx.doi.org/10.1111/1756-185X.14123DOI Listing
May 2021

The microbiome and systemic sclerosis: A review of current evidence.

Best Pract Res Clin Rheumatol 2021 Apr 10:101687. Epub 2021 Apr 10.

Department of Rheumatology & Immunology, Singapore General Hospital, Singapore; Duke-NUS Medical School, Singapore. Electronic address:

Systemic sclerosis (SSc) is characterized by immune dysregulation, vasculopathy, and fibrosis of multiple organs. The gastrointestinal (GI) tract is the most common internal organ manifestation, which contributes to significant morbidity and mortality in patients with SSc. Emerging reports have identified unique microbial taxa alterations in the GI microbiome of patients with SSc as compared to healthy controls (HC). These taxa alterations include differences at the phyla (e.g., Bacteroidetes) and genera (e.g., Bacteroides, Clostridium, Faecalibacterium, and Lactobacillus) level. In addition, some genera have been associated with more severe GI symptoms (e.g., Prevotella and Akkermansia). This review summarizes the current evidence on factors influencing the GI microbiome, GI microbiome alterations in SSc as compared to HC, and in SSc subgroups according to disease manifestations. Current exploration in therapeutic interventions that target the GI microbiome is discussed.
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http://dx.doi.org/10.1016/j.berh.2021.101687DOI Listing
April 2021

Improving sensitivity of the connective tissue disease screening questionnaire: A comparative study of various scoring methods.

Lupus 2021 Jan 22;30(1):35-44. Epub 2020 Oct 22.

Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore.

Objectives: Early detection of autoimmune rheumatic diseases is crucial given their high morbidity and mortality and short window of opportunity to improve patient outcomes. Self-administered screening questionnaires such as the connective tissue disease screening questionnaire (CSQ) have been shown to promote early detection of autoimmune rheumatic diseases. However, optimal scoring of screening questionnaires may differ with prevalence of clinical features and changes in classification criteria. We compared the performance of 3 scoring methods for the CSQ for early detection of autoimmune rheumatic diseases in a multi-ethnic Asian population.

Methods: Patients who were newly referred for evaluation of possible autoimmune rheumatic diseases were invited to answer the cross-culturally adapted CSQ. Detection of autoimmune rheumatic diseases using 1) the original CSQ scoring, 2) a modified CSQ scoring and 3) a scoring based on current classification criteria, were compared to classification of autoimmune rheumatic diseases by classification criteria.

Results: Of 819 participants, 85 were classified as having autoimmune rheumatic diseases screened for by the adapted CSQ. The original CSQ scoring yielded relatively lower sensitivities in detecting both any and individual autoimmune rheumatic diseases (67% and 20-57%, respectively) compared to the modified CSQ scoring (81% and 60-73%, respectively) and the scoring based on current classification criteria (89% and 50-88%, respectively).

Conclusion: The adapted CSQ with the classification criteria-based scoring achieved relatively high sensitivities in detecting autoimmune rheumatic diseases, suggesting this could be employed as the first step in population screening.
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http://dx.doi.org/10.1177/0961203320966378DOI Listing
January 2021

Systemic Sclerosis Perturbs the Architecture of the Immunome.

Front Immunol 2020 6;11:1602. Epub 2020 Aug 6.

Translational Immunology Institute, Singhealth/Duke-NUS Academic Medical Centre, Singapore, Singapore.

Systemic sclerosis (SSc) is an autoimmune disease characterized by excessive fibrosis of skin and internal organs, and vascular dysfunction. Association of T and B cell subsets has been reported in SSc; however, there is lack of systematic studies of functional relations between immune cell subsets in this disease. This lack of mechanistic knowledge hampers targeted intervention. In the current study we sought to determine differential immune cell composition and their interactions in peripheral blood of SSc patients. Mononuclear cells from blood of SSc patients ( = 20) and healthy controls ( = 10) were analyzed by mass cytometry using a 36-marker (cell surface and intracellular) panel. Transcriptome analysis (m-RNA sequencing) was performed on sorted T and B cell subsets. Unsupervised clustering analysis revealed significant differences in the frequencies of T and B cell subsets in patients. Correlation network analysis highlighted an overall dysregulated immune architecture coupled with domination of inflammatory senescent T cell modules in SSc patients. Transcriptome analysis of sorted immune cells revealed an activated phenotype of CD4 and mucosal associated invariant T (MAIT) cells in patients, accompanied by increased expression of inhibitory molecules, reminiscent of phenotype exhibited by functionally adapted, exhausted T cells in response to chronic stimulation. Overall, this study provides an in-depth analysis of the systemic immunome in SSc, highlighting the potential pathogenic role of inflammation and chronic stimulation-mediated "functional adaptation" of immune cells.
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http://dx.doi.org/10.3389/fimmu.2020.01602DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7423974PMC
April 2021

Mortality and hospitalization outcomes of interstitial lung disease and pulmonary hypertension in the Singapore systemic sclerosis cohort.

Semin Arthritis Rheum 2020 06 13;50(3):473-479. Epub 2019 Nov 13.

Department of Rheumatology and Immunology, Singapore General Hospital, Outram Road, Singapore 169608, Singapore. Electronic address:

Objectives: We compared mortality and hospitalization rates in four groups of patients with systemic sclerosis (SSc) [isolated pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD), concomitant ILD-pulmonary hypertension (PH), and no/mild pulmonary involvement].

Methods: In the Systemic Sclerosis Cohort Singapore (SCORE), ILD was diagnosed by HRCT and significant ILD was defined by forced vital capacity <70% predicted. Patients were classified as PAH if echocardiographic systolic pulmonary artery pressure (sPAP) ≥50 mmHg or right heart catheterization (RHC) mean PAP ≥25 mmHg. Multivariable regression analyses were performed to determine factors associated with mortality and hospital admissions per year. Cox proportional hazard model was used to analyze survival.

Results: Of 490 SSc patients, 50 patients had PAH, 92 patients had ILD and 43 patients had ILD-PH. Of 93 patients with PAH or ILD-PH, 56 were based on echocardiography and 37 on RHC. Patients with ILD-PH (HR 3.77, 95% CI: 2.05-6.93) had the highest risk of death, followed by PAH (HR 3.03, 95% CI: 1.60-5.76) and ILD (HR 1.84, 95% CI: 1.04-3.28). After adjustment for confounders, PAH (HR 2.39, 95% CI: 1.13-5.07) remained independently associated with mortality, but not ILD-PH or ILD. Other factors associated with mortality were male gender, age at SSc diagnosis, malabsorption and digital ulcer/ gangrene. Increased hospitalization rate was associated with renal crisis, right heart failure and PAH medications, but not SSc groups.

Conclusion: PAH is an independent risk factor of mortality in SSc. Increased hospitalization rate was not associated with SSc groups. Other factors associated with increased mortality and hospital admissions were identified.
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http://dx.doi.org/10.1016/j.semarthrit.2019.11.005DOI Listing
June 2020

Cross-cultural adaptation of the Hamilton axial spondyloarthritis questionnaire and development of a Chinese version in a multi-ethnic Asian population.

Int J Rheum Dis 2019 Sep 27;22(9):1652-1660. Epub 2019 Jun 27.

Department of Rheumatology and Immunology, Singapore General Hospital, Singapore City, Singapore.

Objective: To cross-culturally adapt the Hamilton axial spondyloarthritis (axial SpA) screening questionnaire and develop a Chinese version for use in a multi-ethnic Asian population in Singapore.

Methods: Consenting participants newly referred from primary care to a rheumatology specialist outpatient clinic for evaluation of possible axial SpA were studied. The original axial SpA questionnaire was revised based on inputs from cognitive debriefing interviews (CDIs) and discussions with an expert panel of rheumatologists and the developer. Forward and back translations of the adapted English version were also reviewed by the expert panel and the developer. The common translation produced was tested in CDIs with Chinese-speaking participants. Adapted English and Chinese versions were pilot-tested in a separate group of similar participants.

Results: Participants were recruited for English (n = 25) and Chinese CDIs (n = 15, relatively older and less frequently presented with axial SpA symptoms), respectively. Alternative terms and explanatory notes were added to difficult medical terms to improve the understandability of the adapted English version. English medical terms were retained in the Chinese version. Pilot-testing of the adapted axial SpA questionnaire was performed on 116 participants, all of whom reported ease of comprehension with both adapted versions. Only one participant was diagnosed with axial SpA, who also scored positive on the adapted axial SpA questionnaire.

Conclusion: The adapted axial SpA questionnaire demonstrated good sensitivity in the pilot-testing and appears to be a promising tool for facilitating early identification of axial SpA cases in the multi-ethnic Asian population.
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http://dx.doi.org/10.1111/1756-185X.13645DOI Listing
September 2019

A double-blind randomized placebo-controlled trial of probiotics in systemic sclerosis associated gastrointestinal disease.

Semin Arthritis Rheum 2019 12 23;49(3):411-419. Epub 2019 May 23.

Department of Rheumatology and Immunology, Singapore General Hospital, The Academia, Level 4, 20 College Road 169856, Singapore; Duke-National University of Singapore Medical School, 8 College Road 169857, Singapore.

Objective: Assess whether treatment with probiotics improve gastrointestinal symptoms in patients with systemic sclerosis (SSc).

Methods: In this double-blind randomized placebo-controlled parallel-group phase II trial, SSc subjects with total score ≥ 0.1 on a validated SSc-specific gastrointestinal tract (GIT) questionnaire were randomized (1:1) to receive 60 days of high dose multi-strain probiotics (Vivomixx® 1800 billion units/day) or identical placebo, followed by an additional 60 days of probiotics in both groups. Between group differences in GIT score change were assessed after 60 days (primary outcome, time-point T1) and 120 days (secondary outcome, time-point, T2) by an intention-to-treat approach. Stool samples at three time-points were subjected to 16S next generation sequencing.

Results: Forty subjects were randomized to placebo-probiotics (n = 21) or probiotics-probiotics (n = 19). At T1, no significant improvement was observed between the two groups, reported as mean ± SE for total GIT score (placebo 0.14 ± 0.06 versus probiotics 0.13 ± 0.07; p = 0.85) or its subdomains. At T2, whilst there was no significant improvement in total GIT score (placebo-probiotics -0.05±0.06; probiotics-probiotics -0.18 ± 0.07; p = 0.14), there was significant improvement of GIT-reflux in the probiotic group (-0.22 ± 0.05 versus placebo-probiotics 0.05 ± 0.07; p = 0.004). Subjects on probiotics exhibited increasing stool microbiota alpha diversity compared to the placebo-probiotics group. Adverse events (AEs) were mild, with similar proportion of subjects with AEs and serious AEs in both groups.

Conclusion: Whilst there was no clear improvement in overall GI symptoms after 60 days, we observed significantly improved GI reflux after 120 days of probiotics. The trial confirmed safety of multi-strain probiotics in SSc patients.

Trial Registration: Clinicaltrials.gov; NCT01804959.
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http://dx.doi.org/10.1016/j.semarthrit.2019.05.006DOI Listing
December 2019

Cross-cultural adaptation of the connective tissue disease screening questionnaire and development of a Chinese version in a multi-ethnic Asian population.

Clin Rheumatol 2019 Sep 29;38(9):2383-2397. Epub 2019 Apr 29.

Department of Rheumatology and Immunology, Singapore General Hospital, Academia Building, Level 4, 20 College Road, Singapore, 169856, Singapore.

Objectives: To cross-culturally adapt the Connective Tissue Disease (CTD) Screening Questionnaire (CSQ) in a multi-ethnic Asian population in Singapore.

Methods: An expert panel of accredited rheumatologists evaluated the content validity of the original CSQ. Consenting participants newly referred from primary care to a rheumatology specialist outpatient clinic for evaluation of possible CTDs were studied. Cognitive debriefing interviews (CDIs) using the original CSQ were conducted with English-speaking participants, with modifications made based on their inputs and in discussion with a second expert panel (rheumatologists and the CSQ developers). Forward and back translations of the adapted English version were reviewed by the second expert panel. The common translation produced was tested in CDIs with Chinese-speaking participants. Adapted English and Chinese versions were pilot tested in a separate group of newly referred patients.

Results: Content validity of the original CSQ was confirmed by the expert panel. A total of 30 and 15 participants were recruited for English and Chinese CDIs, respectively. Alternative terms and explanatory notes were added to difficult medical terms in the adapted English CSQ. A further explanatory note was added to one difficult item, and English medical terms were retained in the Chinese version. Pilot testing of the adapted CSQ was performed on 116 participants, which exhibited an overall sensitivity and specificity of 71% and 58%, respectively, in identifying CTDs.

Conclusions: The adapted CSQ demonstrated satisfactory sensitivity in the pilot testing and appears to be a promising tool for facilitating early identification of CTDs in the multi-ethnic Asian population.

Key Points: • Early identification and management of patients with CTDs is crucial given their high disease burden and short "windows of opportunity." • High reliability and validity of original CSQ and its cross-culturally adapted versions have been reported; however, the CSQ has not been validated in Southeast Asia where CTDs are associated with higher morbidity and mortality compared to other countries. • Our cross-culturally adapted CSQ demonstrated satisfactory sensitivity in identifying CTDs in the multi-ethnic Asian population.
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http://dx.doi.org/10.1007/s10067-019-04567-5DOI Listing
September 2019

F-FDG PET-MRI with T1 MOLLI mapping to detect systemic sclerosis bowel inflammation and fibrosis.

Eur J Radiol 2018 Aug 26;105:289-295. Epub 2018 Jun 26.

Duke-National University of Singapore Medical School, Singapore; Department of Rheumatology and Immunology, Singapore General Hospital, Singapore. Electronic address:

Background: Systemic sclerosis-associated gastrointestinal tract involvement (SSc-GIT) is an independent predictor of 2-year mortality in early SSc. Availability of non-invasive investigations will facilitate early diagnosis and monitoring.

Hypothesis: We investigate the role of F-FDG-PET-MRI in SSc-GIT, hypothesizing that i) higher bowel FDG-PET uptake, a surrogate biomarker for inflammation, distinguishes healthy bowel from inflamed SSc-GIT; ii) MRI T1-MOLLI mapping, a surrogate biomarker for cardiac fibrosis, distinguishes healthy bowel from fibrotic SSc-GIT.

Methods: In this prospective study, 16 SSc patients and 15 healthy controls were recruited. All SSc patients and 5 controls underwent PET-MRI (with T1-MOLLI mapping) on a Siemens 3T mMR; 10 controls underwent MRI without PET. Manual segmentation of the large and small bowels was performed jointly by two trained analysts in order to report T1 and PET values. Control dataset was used to assess normal healthy range. Mean T1 values, mean Tissue-to-Background (TBR) PET values, as well as amount of supposedly abnormal bowel (measured using the healthy ranges) was compared using Student's t-test and Cohen's d effect size.

Results: Mean T1 values in large (1113 ± 182 ms vs 856 ± 176 ms; p-value < 0.001) and small bowel (1331 ± 239 ms vs 1169 ± 118 ms; p = 0.02) were higher in SSc patients than controls. 87.5% of the SSc patients' bowel had at least a grade 3 segmental FDG-PET uptake, while no controls showed more than a grade 2 segmental uptake. Patients had higher large bowel mean PET TBR (1.12 ± 0.22) than controls (0.82 ± 0.20, p = 0.02). Using PET and T1 thresholds defined using the control PET-MR data, the percentage of supposedly healthy (non-fibrotic and non-inflamed) tissue was significantly lower in SSc patients (81.1 ± 13.1%) than controls (95.7 ± 3.1%, p = 0.03) for the large bowel.

Conclusion: Our novel study of FDG-PET-MRI in SSc-GIT demonstrated promising results in non-invasively evaluating concurrently bowel inflammation and fibrosis.
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http://dx.doi.org/10.1016/j.ejrad.2018.06.022DOI Listing
August 2018

Validation of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0 in English- and Chinese-speaking patients in a multi-ethnic Singapore systemic sclerosis cohort.

Clin Rheumatol 2017 Jul 5;36(7):1643-1648. Epub 2017 Jan 5.

Department of Rheumatology and Immunology, Singapore General Hospital, The Academia, Level 4, 20 College Road, Singapore, 169856, Singapore.

The aim of this study was to (1) translate the Gastrointestinal Tract Instrument (GIT) 2.0 from English to Chinese and (2) validate both versions in a multi-ethnic systemic sclerosis cohort in Singapore (SCORE). The English GIT2.0 was translated to Chinese using a standard forward-backward translation approach. Psychometric evaluation of the GIT2.0 included internal consistency reliability (using Cronbach's alpha), test-retest reliability (using intra-class correlation coefficient (ICC)), scale level factor analysis, and construct validity (using Spearman correlation) against the modified Scleroderma Health Assessment Questionnaire (S-HAQ) and the SF-36 v2. Most of the patients were females (88.6%) and Chinese (78.2%), with mean (SD) age of 51.0 (13.0) years and median disease duration of 4.5 years. We administered English (n = 146) and Chinese (n = 74) GIT2.0. The mean (SD) total GIT score was 0.29 (0.37). There was good internal consistency (Cronbach's alpha >0.70 for all subscales) and good test-retest reliability for the scale and all subscales (ICC 0.71-0.92) except for "diarrhoea" (ICC = 0.54). Our hypothesised a priori construct validity was supported by moderate correlations between the total GIT score and S-HAQ GI subscale (r = 0.446), and the social functioning subscale and SF36v2 role-social domain (r = 0.337), and weak-to-moderate correlation between the emotional subscale and SF-36v2 role-emotional (r = 0.295) and mental health (r = 0.298) domains and mental component summary (r = 0.356). Exploratory factor analysis of the seven subscales yielded a two-factor solution explaining 69.63% of the total variance. This study provides evidence for the reliability and validity of the English and Chinese GIT2.0 to be used in Singapore for research and routine practice.
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http://dx.doi.org/10.1007/s10067-016-3529-xDOI Listing
July 2017

Comparison of the Charlson Comorbidity Index derived from self-report and medical record review in Asian patients with rheumatic diseases.

Rheumatol Int 2015 Dec 10;35(12):2005-11. Epub 2015 Jun 10.

Department of Rheumatology and Immunology, Singapore General Hospital, The Academia, 20 College Road, Singapore, Singapore.

The aim of the study was to compare the agreement between self-report Charlson Comorbidity Index (SR-CCI) and the medical record-based CCI (MR-CCI) and to examine the impact of both instruments on health-related quality of life (HRQoL) amongst Asian patients with rheumatic diseases. This cross-sectional study surveyed a convenience sample of patients seen at rheumatology specialty outpatient clinics. Patients completed the SR-CCI and Short Form 36, while two research assistants completed the MR-CCI. Item-level agreement between the SR-CCI and MR-CCI was evaluated using kappa coefficients. Adjusted linear regression models evaluated the independent effect of the SR-CCI/MR-CCI on HRQoL. The study included 301 patients (median age 51, range 21-79, 61.5 % female, 68.8 % Chinese, 17.6 % Indian, 6.0 % Malay). Kappa statistics for cerebrovascular disease (0.433), chronic pulmonary disease (0.509), connective tissue disease/rheumatoid arthritis (0.506), ulcer disease (0.461), and tumour (0.541) reflected moderate agreement between the SR-CCI and MR-CCI (all p < 0.0001). There was substantial agreement in the reporting of diabetes (0.764, p < 0.0001) but poor/fair agreement for that of myocardial infarction (0.359, p < 0.0001) and diabetes with end-organ damage (0.189, p = 0.0002). Increases in SR-CCI were associated with significant reductions in both physical (β coefficient -2.56, p < 0.0001) and mental HRQoL (β coefficient -1.24, p = 0.044). However, such associations were not observed with the MR-CCI. The SR-CCI demonstrated moderate concordance with the MR-CCI, and the SR-CCI but not MR-CCI scores were associated with lower HRQoL. Assessment of comorbidities amongst rheumatology patients remains complex, and more efficient methods of quantifying these conditions are needed for clinical and research purposes.
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http://dx.doi.org/10.1007/s00296-015-3296-zDOI Listing
December 2015

Disease patterns of rheumatology outpatients seen in a tertiary hospital serving a multi-ethnic, urban Asian population in Singapore.

Int J Rheum Dis 2013 Jun 17;16(3):273-8. Epub 2012 Dec 17.

Department of Rheumatology and Immunology, Singapore General Hospital, Singapore.

Aims: To describe the spectrum of diseases seen in an outpatient setting in the Singapore General Hospital, the largest tertiary referral centre in Singapore.

Methods: In this cross-sectional study, medical records of patients scheduled for an appointment at the rheumatology specialist outpatient clinics over a 4-month period (10 August 2010-31 December 2010) were reviewed. Primary diagnoses documented by the attending physician at the latest visit were recorded.

Results: Among 4180 patients (29.5% male; mean [SD] age: 53.5 [15.1] years; 77.0% Chinese, 8.0% Malay, 8.8% Indian and 6.2% others), the spectrum of diseases seen was as follows [disease - definite n (%), probable n (%)]: Arthritis: rheumatoid arthritis - 958 (22.9%), 68 (1.6%); osteoarthritis - 452 (10.8%), 39 (0.9%); crystal arthritis - 417 (10.0%), 18 (0.4%); spondyloarthritis - 227 (5.4%), 61 (1.5%); psoriatic arthritis - 158 (3.8%), 9 (0.2%); other inflammatory arthritis - 153 (3.7%), 94 (2.2%); Connective tissues diseases: systemic lupus erythematosus - 412 (9.9%), 26 (0.6%); vasculitis - 105 (2.5%), 22 (0.5%); Sjögren's syndrome - 81 (1.9%), 32 (0.8%); overlap syndromes - 73 (1.8%); scleroderma - 50 (1.2%), 4 (0.1%); undifferentiated connective tissue diseases - 45 (1.1%), 106 (2.5%); myositis - 41 (1.0%), 12 (0.3%); antiphospholipid syndrome - 22 (0.5%), 7 (0.2%); polymyalgia rheumatica - 16 (0.4%); Others: soft tissue rheumatism - 155 (3.7%); osteoporosis - 61 (1.5%); other non-rheumatologic conditions - 189 (4.5%); other rheumatologic conditions - 67 (1.6%).

Conclusion: Rheumatoid arthritis, osteoarthritis and crystal arthritis were the three most common rheumatological diseases seen in a tertiary referral centre serving a multi-ethnic urban Asian population in Singapore.
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http://dx.doi.org/10.1111/1756-185x.12016DOI Listing
June 2013

Recurrent aortic aneurysms following thoracic aortic stent-graft repair in a patient with Cogan syndrome.

J Endovasc Ther 2006 Dec;13(6):779-82

Department of Cardiothoracic Surgery, National Heart Centre, 17 Third Hospital Avenue, Singapore 168752.

Purpose: To report the need for multiple surgical interventions to treat recurrent aortic aneurysms in a patient with Cogan syndrome.

Case Report: A 17-year-old Chinese man with clinical Marfanoid features had a left common carotid artery pseudoaneurysm electively repaired with an autologous saphenous vein graft. Four months later, he presented with acute chest pain. Computed tomography (CT) revealed a 1-cm pseudoaneurysm at the mid descending aorta; a 24 x 100-mm Talent stent-graft was implanted to exclude the pseudoaneurysm. He was also found to have increasing left-sided hearing loss. A month later, the patient was re-admitted with vertigo and keratitis, which were treated appropriately. Nine months following stent-graft insertion, he was admitted with acute hemoptysis. Urgent CT showed a rupture at the proximal end of the stent-graft, with hemorrhage into the lung parenchyma. In an emergent procedure, the stent-graft was removed, and the descending thoracic aorta was repaired. Intraoperatively, a large pseudoaneurysm was found arising from the proximal part of the stented aorta, which appeared thickened. His postoperative recovery was uneventful. Nine months after the thoracotomy, a routine CT revealed an aneurysm at the distal descending thoracic aorta. On re-thoracotomy, a de novo saccular aneurysm was found 2.5 cm from the distal anastomosis. The affected segment was replaced with a Dacron graft. The distal aorta appeared thickened and edematous; histology confirmed aortitis. The patient was subsequently diagnosed with Cogan syndrome and given corticosteroids and methotrexate. There is no evidence of recurrence at nearly 2 years after the last intervention.

Conclusion: This case highlights the pitfalls of stent-graft repair in a patient with presumed connective tissue disease.
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http://dx.doi.org/10.1583/06-1822MR.1DOI Listing
December 2006