Displaying publications 1 - 20 of 41 in total

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  1. Sridharan R, Ngiu CS, Shaharir SS, Said MS
    BMJ Case Rep, 2015;2015.
    PMID: 26677159 DOI: 10.1136/bcr-2015-213220
    Spondyloarthropathy (SpA) is a group of inflammatory conditions that include spondylitis, sacroiliitis, asymmetrical peripheral arthritis and enthesitis. This condition is known as juvenile SpA when the diagnosis is made in patients up to 16 years of age. Enthesitis is a highly specific feature that occurs more often in juvenile SpA than in the adult form. In contrast to adult onset SpA, the initial manifestation of juvenile SpA rarely presents as inflammatory back pain. Peripheral arthritis is the more common presenting feature. We report a case of a 12-year-old boy who presented with a 1-year history of progressive low back pain, gluteal pain and thigh pain. There were no clinical symptoms of arthropathy of the distal extremities. MRI of the whole spine was performed twice, which, unfortunately, was unyielding. Finally, MRI of the sacroiliac joints revealed asymmetric sacroiliitis as well as enthesitis of the hips and pelvis. Further laboratory data showed negative rheumatoid factor and positive human leucocyte antigen (HLA) B27. A diagnosis of juvenile SpA with sacroiliitis and enthesitis was made. The imaging characteristics of juvenile SpA are highlighted.
  2. Shaharir SS, Mohamed Said MS, Kong NC
    Reumatismo, 2012;64(6):341-9.
    PMID: 23285477 DOI: 10.4081/reumatismo.2012.341
    OBJECTIVES: To investigate the prevalence of thickened carotid intima media thickness (CIMT) and its associated risk factors in patients with lupus nephritis (LN) who were in remission.
    METHODS: This was a cross sectional study in which consecutive LN patients who were in remission and attending our Nephrology/SLE Clinic were included. Their demographic profile, traditional cardiovascular risk factors and treatment medications were evaluated by clinical interview and review of medical records. Carotid intima media thickness (CIMT) was measured using B Mode carotid ultrasonography. CIMT was considered to be abnormally thickened if it exceeded the 75th percentile matched for age-and sex-matched normal controls. The associated factors for thickened CIMT were examined.
    RESULTS: A total of 39 patients with a mean remission duration of 29 ± 24.3 months and on a mean prednisolone dose of 9.10 ± 7.83 mg daily completed the study. Six patients (15.4%) had thickened CIMT. On univariate analysis, male gender, patient age, older age at diagnosis, higher serum CRP levels, greater proteinuria and higher mean cumulative azathioprine dose were associated with thickened CIMT (P<0.05). Lower mean cumulative doses of cyclosporine A (CyA) and mycophenolic acid (MPA) (P<0.05) each were associated with thickened CIMT. Using regression analysis, the associated factors of CIMT were older age at diagnosis and proteinuria.
    CONCLUSIONS: Lupus factors particularly age at diagnosis and proteinuria were the associated factors of thickened CIMT. Larger prospective trials are indicated to confirm our findings.
  3. Ismail E, Nofal OK, Sakthiswary R, Shaharir SS, Sridharan R
    PLoS One, 2016;11(4):e0153752.
    PMID: 27105431 DOI: 10.1371/journal.pone.0153752
    OBJECTIVE: Interleukin-1 receptor antagonist (IL-1Ra) acts as an inhibitor of IL-1; which is one of the culprit cytokines in rheumatoid arthritis (RA). Although +2018 polymorphism of IL-1Ra has been implicated in the pathogenesis of RA, its importance remains poorly understood. Hence, the purpose of this study was to determine the clinical significance of interleukin-1 receptor antagonist (IL-1Ra) +2018 polymorphism in RA.
    METHODS: Polymerase chain reaction (PCR) and sequencing were used to determine the genotypes of the IL-1Ra +2018 for 77 RA patients and 18 healthy controls. All RA patients were assessed for the disease activity score that includes 28 joints (DAS28) and radiographic disease damage based on Modified Sharp Score (MSS).
    RESULTS: The frequency of the T/T and C/T genotypes did not differ significantly (p = 0.893) between the RA patients and the controls. The C/T genotype had significantly higher mean disease activity (DAS 28) and disease damage (MSS) scores with p values of 0.017 and 0.004, respectively. Additionally, the ESR (erythrocyte sedimentation rate), CRP (C-reactive protein), the number of swollen and tender joints were higher for the C/T individuals. On multivariate analysis the CRP, swollen joint count and MSS remained significant with the following p values i.e. 0.045, 0.046 and less than 0.05.
    CONCLUSIONS: C/T genotype of IL-1Ra +2018 prognosticates more aggressive disease in RA.
    Study site: Outpatient clinic, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
  4. Shaharir SS, Jamil A, Chua SH, Arumugam M, Rosli N
    Dermatol Ther, 2020 11;33(6):e14021.
    PMID: 32677247 DOI: 10.1111/dth.14021
    Hidradenitis suppurativa (HS) is a debilitating chronic inflammatory skin disease. Biological therapy has revolutionized it's the treatment. Paradoxical HS occur with various biological and targeted agents. We report a patient with juvenile rheumatoid arthritis who developed HS after 6 months of tofacitinib therapy. A comprehensive literature review identified 43 cases of paradoxical HS among patients on biological and targeted agents. Pooled analysis of the cases showed Crohn's disease 18(41.8%) and RA 9(20.9%) as commonest indications for biological therapy. Adalimumab 20(46.5%) followed by infliximab 9(20.9%) were the commonest offending agents. Duration of biological treatment prior to HS manifestation was 12(1-120) months. Smoking 21(48.8%) and overweight or obese 20(46.5%) were most frequent HS risk factors. Fourteen (32.6%) patients had a second paradoxical event, 11(25.6%) developed psoriasis and 4(9.3%) Crohn's disease. Presence of ≥1 risk factor for HS, continuation of the implicated biological agent and occurrence of more than one paradoxical event were factors associated with poor paradoxical HS outcome.
  5. Sakthiswary R, Omimah KJN, Endom I, Shaharir SS, Sridharan R
    Medicine & Health, 2016;11(2):209-217.
    MyJurnal
    The search for novel biomarkers has taken centre stage in the past decades of research in Rheumatoid Arthritis (RA). The purpose of the present study was to determine the correlation of serum matrix metalloproteinase-3 (MMP-3) with disease activity, joint damage and functional disability in patients with RA. We consecutively recruited RA patients who were under follow-up at the Universiti Kebangsaan Malaysia Medical Centre (UKMMC). Information on the RA disease characteristics were obtained from the medical records and all RA patients were
    assessed for DAS28 (disease activity score based on 28 joints) and Stanford Health Assessment Questionnaire (HAQ) 8-item Disability Index (HAQ-DI). The hand radiographs of the RA patients were assessed for joint damage using the Modified Sharp Score (MSS). Serum MMP-3 levels from RA patients and healthy controls were measured using the ELISA method. We recruited a total of 77 RA patients and 18 healthy controls. The serum MMP-3 levels were significantly higher among the RA patients (p<0.05). There were significant correlations between the serum MMP3 levels and MSS (r =0.327) and HAQ-DI (r=0.256), both p<0.05. The mean serum MMP levels in RA patients with radiographic joint erosions was significantly higher than in patients without erosions (p<0.05). Likewise, the subjects with significant functional impairment i.e HAQ-DI ≥1; had significantly higher mean MMP-3 levels compared to RA patients without significant disability (p<0.05). Using multivariate analysis, HAQ-DI remained the independent predictor of serum MMP-3 in RA patients. Serum MMP-3 is a potential biomarker and predictor of radiographic joint damage and functional disability in RA.
    Keywords: acquired joint deformity, matrix metalloproteinases, rheumatoid arthritis
  6. Nor Hashimah AMM, Sakthiswary R, Shaharir SS, Wahab A
    Arch Rheumatol, 2019 Dec;34(4):387-394.
    PMID: 32010887 DOI: 10.5606/ArchRheumatol.2019.7243
    Objectives: This study aims to compare the serum pyridinoline (Pyd) levels between rheumatoid arthritis (RA) patients and healthy controls and to determine the correlation of serum Pyd levels with radiographic joint erosions.

    Patients and methods: Serum samples were obtained from 48 patients with RA (9 males, 39 females; mean age 60.5 years; range 54 to 64 years) and 48 healthy controls (9 males, 39 females; mean age 57.5 years; range, 47 to 65 years). The enzyme-linked immunosorbent assay method was used for quantitative analysis of serum Pyd. Besides, all RA patients were assessed for joint damage based on modified Sharp score, disease activity based on disease activity score in 28 joints and functional capacity based on health assessment questionnaire-disability index.

    Results: The median serum Pyd levels were significantly higher among the RA patients (110.20 ng/mL [92.30-120.64]) compared to the controls (98.22 ng/mL [85.54-111.41]); p<0.05. RA patients with erosive disease had significantly higher serum Pyd levels (p=0.024). There was a significant positive correlation between serum Pyd levels and joint erosion score (r=0.285, p=0.049). The serum Pyd levels had no demonstrable association with disease activity or functional capacity. Steroid therapy did not appear to influence the levels of serum Pyd.

    Conclusion: Rheumatoid arthritis patients had significantly higher levels of serum Pyd compared to healthy controls. The serum Pyd levels had significant correlation with radiographic joint erosions which reflected disease damage.

  7. Kusyairi KA, Gendeh BS, Sakthiswary R, Shaharir SS, Haizlene AH, Yusof KH
    Lupus, 2016 Apr;25(5):520-4.
    PMID: 26657735 DOI: 10.1177/0961203315622279
    The purpose of this study was to determine the spectrum of nasal involvement in systemic lupus erythematosus (SLE) and its association with the disease activity of SLE based on the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). This was a cross-sectional and observational study involving 73 stable SLE patients. All subjects were evaluated for the SLEDAI scores and had nasal endoscopic examination. The most commonly reported symptom was nasal congestion (31.5%) followed by nasal itchiness (26.0%), runny nose (20.5%) and nasal dryness (19.2%). Almost half (42.9%) of the subjects had nasal mucosal abnormalities, which included mucositis, crusting, ulceration, bifid middle turbinate, septal spur, Jacobson's organ, deviated nasal septum, bilateral inferior turbinate hypertrophy, everted uncinate process, nasopharynx cleft and torus palatinus. The median SLEDAI score for subjects with nasal symptoms was significantly higher than subjects without nasal symptoms (p 
  8. Shaharir SS, Mustafar R, Mohd R, Mohd Said MS, Gafor HA
    Clin Rheumatol, 2015 Jan;34(1):93-7.
    PMID: 25373448 DOI: 10.1007/s10067-014-2802-0
    Arterial hypertension (HPT) burden up to two third of systemic lupus erythematosus (SLE) patients and contributes to accelerated atherosclerosis and cardiovascular (CV) risk. We aim to determine the prevalence of HPT among lupus nephritis (LN) patients who were in complete remission (CR) for a minimum of 6 months, with estimated glomerular filtration rate (eGFR) of >60 mL/min/1.73 m(2). This is a cross-sectional study of 64 LN patients who attended Nephrology/SLE Clinic at The National University of Malaysia Medical Centre (UKMMC). Persistent hypertension (blood pressure (BP) ≥140/90 mmHg for at least two occasions), CR for a minimum of 6 months and eGFR of >60 mL/min/1.73 m(2) were identified. Univariate and multivariate analyses were performed to determine the demographic and disease characteristics associated with HPT. Thirty-four of them (53.1 %) were hypertensive. Persistent HPT was associated with disease duration, acute kidney injury and high BP at the onset of LN, longer duration interval to achieve CR, number of relapses and cyclosporine A (CyA) use. There were no associations between histological classes, nephrotic range proteinuria, body mass index and waist circumference with HPT. Factors independently associated with HPT were disease duration OR 1.06 [95 %CI (0.91-1.24)], longer duration interval to achieve CR OR 1.104 [95 %CI (1.02-1.19)], number of relapses OR 2.53 [95 % CI (1.01-6.3)] and CyA use OR 5.3 [95 % CI (1.14-23.9)]. The prevalence of HPT among LN is high despite in remission. Aggressive treatment is important to achieve early CR and to prevent relapses.
    Study site: Nephrology/SLE clinics, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
  9. Sakthiswary R, Shaharir SS, Mohd Said MS, Asrul AW, Shahril NS
    Int J Rheum Dis, 2014 Nov;17(8):872-7.
    PMID: 25292482 DOI: 10.1111/1756-185X.12443
    AIM: The main objective of this study is to elucidate the role of immunoglobulin A (IgA) rheumatoid factor (RF) in predicting the clinical response to tumour necrosis factor α inhibitors (TNFi) among patients with rheumatoid arthritis (RA).
    METHOD: We recruited all patients with RA who were ever on TNFi for a minimum duration of 3 months at our centre. Based on the European League Against Rheumatism response criteria, subjects were further divided into responders and non-responders. Age-matched RA patients who were on conventional disease-modifying anti-rheumatic drugs and in remission were enrolled as controls. Subjects were tested for quantitative values of IgA, IgM, IgG RF and anti-citrulinated cyclic peptides (CCP). Further, all subjects were assessed for the disease activity score that includes 28 joints (DAS28) and Stanford Health Assessment Questionnaire (HAQ) 8-item Disability Index (HAQ-DI).
    RESULTS: A total of 31 subjects with RA who had received TNFi and 15 controls were enrolled in this study. There was a trend for the non-responders (n = 10) to have higher levels of all isotypes of RF and anti-CCP. However, only the IgA RF and anti-CCP levels were significantly higher in the non-responder group compared to the responders and controls (P = 0.001, P = 0.034, respectively). On multivariate analysis, only the IgA RF remained significant (OR 0.989; 95% CI 0.980-0.999; P = 0.026).
    CONCLUSION: IgA RF is potentially a novel predictor of response to TNFi in RA patients. Testing for pretreatment IgA RF levels could be a reasonable consideration before commencement of TNFi.
    Study site: Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
  10. Shaharir SS, Gafor AH, Said MS, Kong NC
    Int J Rheum Dis, 2015 Jun;18(5):541-7.
    PMID: 25294584 DOI: 10.1111/1756-185X.12474
    OBJECTIVE:
    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease and glucocorticoid is the mainstay of treatment in SLE. The reported incidence of steroid-induced diabetes mellitus (SDM) ranged between 1-53%. We sought to investigate the prevalence and associated factors of SDM in patients with SLE.

    METHODOLOGY:
    A total of 100 SLE patients attending the Nephrology/SLE and Rheumatology Clinic, Universiti Kebangsaan Malaysia Medical Centre (UKMMC) who received corticosteroid treatment were recruited. The diagnosis of diabetes mellitus was based on the 2010 American Diabetes Association's criteria. Prevalent cases of SDM were also included. Statistical analysis was performed to determine the factors associated with SDM.

    RESULTS:
    Thirteen of them (13%) developed SDM, with the median onset of diagnosis from commencement of glucocorticoid treatment being 8 years (range 0.5-21 years). Although only seven Indians were recruited into the study, three of them (42.9%) had SDM compared to Malays (9.3%) and Chinese (12.8%) (P ≤ 0.05). Univariate and multivariate analysis showed that higher numbers of system or organ involvement in SLE, abdominal obesity, hypertriglyceridemia and daily prednisolone of ≥ 1 mg/kg/day were the important associated factors of SDM (P ≤ 0.05). Meanwhile, hydroxychloroquine (HCQ) use was associated with reduced SDM prevalence (P < 0.05).

    CONCLUSION:
    The prevalence of SDM among SLE patients was 13% and Indians were more prone to develop SDM compared to other races. Higher numbers of system involvement, abdominal obesity, hypertriglyceridemia and the use of oral prednisolone of ≥ 1 mg/kg/day were associated with SDM, while HCQ use potentially protects against SDM.

    © 2014 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

    KEYWORDS:
    SLE drug treatment; clinical aspects; systemic lupus erythematous
  11. Dalila AS, Mohd Said MS, Shaharir SS, Asrul AW, Low SF, Shamsul AS, et al.
    Kaohsiung J. Med. Sci., 2014 Jul;30(7):337-42.
    PMID: 24924839 DOI: 10.1016/j.kjms.2014.02.010
    The purpose of this study was to compare the serum interleukin (IL)-23 levels between rheumatoid arthritis (RA) patients and healthy controls and to determine the correlation of IL-23 levels with disease activity, joint damage and functional disability in RA. Serum samples were obtained from 45 patients with RA and 45 healthy controls. The enzyme-linked immunosorbent assay method was used for quantitative analysis of IL-23. All the RA patients were assessed for disease activity based on the 28-joint disease activity score, joint damage based on modified Sharp score, and functional ability using the Health Assessment Questionnaire-Disability Index. The mean serum IL-23 level was much higher among the RA patients (24.50 ± 13.98 pg/mL) compared to the controls (5.98 ± 3.40 pg/mL; p < 0.01). There was a significant positive relationship between IL-23 levels and disease activity and questionnaire scores (p = 0.003 and 0.020, respectively). On logistic regression analysis, IL-23 levels were significantly higher in patients with moderate to high disease activity (p = 0.008, odds ratio = 1.073, 95% confidence interval = 1.019-1.130) and patients with significant functional disability (p = 0.008, odds ratio = 1.085, 95% confidence interval = 1.021-1.153). RA patients have significantly higher levels of serum IL-23. The IL-23 levels correlate well with disease activity and functional disability but not with radiographic joint damage.

    Study site: Rheumatology clinic, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM)
  12. Sahari NS, Shaharir SS, Ismail MR, Rajalingham S, Mohamed Said MS
    Mod Rheumatol, 2014 Nov;24(6):920-5.
    PMID: 24645724 DOI: 10.3109/14397595.2014.891497
    OBJECTIVE: To determine the associated factors of subclinical atherosclerosis measured with carotid intima media thickness (CIMT) among rheumatoid arthritis (RA) patients without any overt traditional cardiovascular (CV) risk factors.
    METHODS: Forty RA patients with matched age and gender healthy controls were recruited. Carotid ultrasound was performed to all subjects. CIMT was considered to be abnormally thickened if it was more than the 75th percentile matched for age and sex reference values. Univariate and multivariate analyses were performed to determine the association between the sociodemographics and disease characteristics of RA with thickened CIMT.
    RESULTS: Abnormally thickened CIMT were observed in 11 RA patients (27.5%) and in 4 control subjects (10%), p = 0.04. It was highly prevalent among RA patients with active disease (54.5% vs 17.2%), p = 0.02. Patients with thickened CIMT also tend to have erosive disease, p = 0.06. Seropositive rheumatoid factor (RF) patients also had significantly higher CIMT values as compared with sero-negative patients, p = 0.03. Multivariable logistic regression analysis revealed that active disease was independently associated with thickened CIMT.
    CONCLUSIONS: RA patients are at risk for subclinical atherosclerosis despite absence of traditional CV risk co morbidities and active disease was the independent factor associated with it.
    KEYWORDS: Atherosclerosis; Carotid intima media thickness; Disease activity; Rheumatoid arthritis
    Study site: Rheumatology Clinic, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Kuala Lumpur, Malaysia
  13. Shaharir SS, Ghafor AH, Said MS, Kong NC
    Lupus, 2014 Apr;23(4):436-42.
    PMID: 24399814 DOI: 10.1177/0961203313518624
    INTRODUCTION: Renal involvement is the most common serious complication in patients with systemic lupus erythematosus (SLE).
    OBJECTIVE: The objective of this article is to investigate and determine the associated factors of disease damage among lupus nephritis (LN) patients.
    METHODS: Medical records of LN patients who attended regular follow-up for at least one year in the Nephrology/SLE Clinic, Universiti Kebangsaan Malaysia Medical Centre (UKMMC), were reviewed. Their Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index scores were noted. Univariate analysis and multivariable regression analysis were performed to determine the independent factors of disease damage in LN.
    RESULTS: A total of 150 patients were included and their follow-up duration ranged from one to 20 years. Sixty (40%) LN patients had disease damage (SDI ≥1). In the univariate analysis, it was associated with age, longer disease duration, antiphospholipid syndrome (APS), higher maximum daily oral prednisolone dose (mg/day), lower mean C3 and C4, higher chronicity index and global sclerosis on renal biopsies (p < 0.05). Patients who received early (≤3 months after the SLE diagnosis) hydroxychloroquine (HCQ), optimum HCQ dose at 6.5 mg/kg/day and achieved early complete remission (CR) were less likely to have disease damage (p < 0.05). After adjustment for age, gender, disease duration and severity, multivariable regression analysis revealed that a higher maximum daily dose of oral prednisolone was independently associated with disease damage while early HCQ and CR were associated with lower disease damage.
    CONCLUSION: Higher maximum daily prednisolone dose predicted disease damage whereas treatment with early HCQ and early CR had a protective role against disease damage.
    KEYWORDS: Antiphospholipid syndrome; lupus nephritis; systemic lupus erythematosus

    Study site: Nephrology/SLE Clinic, Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM)
  14. Rajalingham S, Said MS, Shaharir SS, AbAziz A, Periyasamy P, Anshar FM
    BMJ Case Rep, 2011;2011.
    PMID: 22675098 DOI: 10.1136/bcr.08.2011.4675
    Dermatomyositis is a rare rheumatic disease which predominantly affects the muscles and skin requiring a protracted course of immunosuppressants which may predispose the patients to opportunistic infections. A 49-year-old lady was diagnosed to have dermatomyositis in August 2010 based on history, significantly raised creatine kinase level and muscle biopsy findings. She had recurrent admissions due to fever, myalgia and muscle weakness. She had spiking temperature despite high dose steroids, broad-spectrum antibiotics and antifungal agents. This prompted extensive investigation which leads us to the additional diagnosis of disseminated tuberculosis involving the lungs, muscles and bones. This case demonstrates the challenge in controlling the disease activity of dermatomyositis with immunosuppressants in the setting of disseminated tuberculosis.
  15. Shaharir SS, Tumian NR, Yu Lin AB, Abdul Wahid SF
    J Infect Dev Ctries, 2013 Mar;7(3):286-8.
    PMID: 23493009 DOI: 10.3855/jidc.2691
    Tuberculosis is notoriously known to be a great mimicker of other diseases and may cause various haematologic abnormalities, especially with marrow involvement. A 61-year-old man who presented with right empyema and pancytopenia was diagnosed to have disseminated tuberculosis supported by the presence of caseating granuloma with Langhan's giant cells in the marrow and demonstration of acid-fast bacilli in the pleural fluid. Trilineage dysplasia from marrow aspirate was initially attributed to be reactive to the infection. A cytogenetic study was repeated after he showed poor response to a year of anti-tuberculosis treatment. The underlying primary myelodysplastic syndrome was unmasked when his cytogenetics showed trisomy 8. This case report has demonstrated the various haematological manifestations of tuberculosis and highlighted the importance of cytogenetic study in differentiating between primary and secondary myelodysplastic marrow changes.
  16. Shaharir SS, Remli R, Marwan AA, Said MS, Kong NCT
    Lupus, 2013 Apr;22(5):492-6.
    PMID: 23435619 DOI: 10.1177/0961203313478303
    INTRODUCTION:Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disorder which is increasingly recognized to occur in systemic lupus erythematosus (SLE).
    OBJECTIVE: The purpose of this study was to identify the characteristics of SLE patients with PRES and the associated factors of the poor outcome among them.
    METHODS: We investigated SLE patients who developed PRES between 2005-2011 at the Universiti Kebangsaan Malaysia Medical Centre. A comprehensive literature search was done to find all published cases of PRES in SLE. Pooled analysis was conducted to identify the factors associated with poor outcome.
    RESULTS: There were 103 cases of PRES in SLE published in the literature but only 87 cases were included in the analysis in view of incomplete individual data in the remaining cases. The majority of the cases were Asians (74.2%), female (95.4%) with mean age of 26.3 ± 8.8 years. PRES was highly associated with active disease (97.5%), hypertension (91.7%) and renal involvement (85.1%). We found that 79 patients had a full recovery (90.8%) with a mean onset of full clinical recovery in 5.6 ± 4.1 days. On univariate analysis and logistic regression analysis the predictors of poor outcome, defined as incomplete clinical recovery or death, were intracranial hemorrhage, odds ratio (OR) 14 (1.1-187.2), p=0.04 and brainstem involvement in PRES, OR 10.9 (1.3-90.6), p=0.003.
    CONCLUSION: Intracranial hemorrhage and brainstem involvement were the two important predictors of poor outcome of PRES. Larger prospective studies are needed to further delineate the risk of poor outcome among them.
  17. Shaharir SS, Mohamed Said MS, Mohd R, Abdul Cader R, Mustafar R, Abdul Rahman R
    PLoS One, 2019;14(9):e0222343.
    PMID: 31539383 DOI: 10.1371/journal.pone.0222343
    Flare of Systemic Lupus Erythematosus (SLE) may occur during pregnancy and puerperium. We studied the prevalence and factors associated with SLE relapse during pregnancy and post-partum period in a multi-ethnic SLE cohort. Consecutive SLE patients who attended the outpatient clinic were reviewed for previous history of pregnancies in our institution. Patients who had a complete antenatal, delivery, and post-partum follow up were included. Their medical records were retrospectively analysed to assess the disease activity at pre-pregnancy/conception, during antenatal, and post-partum period. Presence of flare episodes during pregnancy and puerperium were recorded. The pregnancy outcomes recorded include live birth, foetal loss, prematurity and intra-uterine growth restrictions (IUGR). Univariate and multivariable logistic regression with generalized estimating equations (GEE) analyses were performed to determine the factors associated with disease relapse and the pregnancy outcomes. A total of 120 patients with 196 pregnancies were included, with a live birth rate of 78.6%. Four (2.0%) were diagnosed to have SLE during pregnancy. The flare rate in pregnancy was 40.1% while post-partum 17.4%. Majority of the relapse in pregnancy occurred in haematological system (62.3%) followed by renal (53.2%), musculoskeletal (22.1%), and mucocutaneous (14.3%). In GEE analyses, active disease at conception was the independent predictor of SLE relapse during and after pregnancy, whereas older maternal age and Malay ethnicity were associated with higher flare during post-partum. HCQ use was significantly associated with reduced risk of flare in univariate analysis but it was no longer significant in the GEE analyses. Presence of disease flare in pregnancy was significantly associated with prematurity. In conclusion, pregnancy in SLE need to be planned during quiescent state as pre-pregnant active disease was associated with disease relapse in both during and after pregnancy. Malay patients had an increased risk of post-partum flare but further larger prospective studies are needed to confirm the association between pregnancies in the different ancestral background.
  18. Abu Bakar F, Shaharir SS, Mohd R, Kamaruzaman L, Mohamed Said MS
    Int J Rheum Dis, 2019 Jun;22(6):1002-1007.
    PMID: 30968556 DOI: 10.1111/1756-185X.13572
    AIM: To determine the prevalence of work disability (WD) among patients with systemic lupus erythematosus (SLE) and its associated factors.

    METHOD: This was a cross-sectional study involving SLE patients aged 18-56 years from Universiti Kebangsaan Malaysia Medical Centre (UKMMC). Employment history was obtained from clinical interviews. WD was defined as unemployment, interruption of employment or premature cessation of employment due to SLE at any time after the diagnosis. SLE disease characteristics, presence of organ damage and Safety of Estrogens in Lupus Erythematosus National Assessment-SLE Disease Activity Index (SLEDAI) flare index were determined from the medical records. Self-reported quality of life (QoL) was performed using the Medical Outcomes Study Short Form-36 (SF-36). Demographic factors, disease characteristics, and QoL were compared between patients with and without WD using statistical analyses.

    RESULTS: A total of 215 patients were recruited and the majority were Malay (60.5%), followed by Chinese (33.5%), Indian (4.5%) and others (n = 4, 1.9%). The prevalence of WD was 43.2% (n = 93) with 22.3% (n = 48) patients were unemployed at the time of study. Over half the patients with WD (n = 51, 54.8%) had onset of disability at <5 years from diagnosis. Patients with WD had significantly lower health-related QoL. The independent factors associated with WD were SLEDAI score at diagnosis, frequency of flare, Systemic Lupus International Collaborating Clinics score, being married, had lower education and lupus nephritis.

    CONCLUSION: We found a high rate of WD in patients with SLE and it was significantly associated with SLE-related factors, in particular higher disease activity, presence of renal involvement and organ damage.

  19. Shaharir SS, Maulana SA, Shahril NS, Mohd R, Mustafar R, Said MSM, et al.
    Lupus, 2020 Sep;29(10):1305-1313.
    PMID: 32660312 DOI: 10.1177/0961203320938871
    BACKGROUND: Despite the improvement in the live birth rate among patients with systemic lupus erythematosus (SLE), they are still at an increased risk of adverse pregnancy outcomes (APOs).

    OBJECTIVE: To determine the prevalence and factors associated with APOs in the multi-ethnic SLE populations in Malaysia.Methodology: This was a retrospective review of the consecutive SLE patients who attended the outpatient clinic in two major rheumatology centres from January 2016 until December 2019 with complete pre-pregnancy, antenatal and intra-partum records. APOs include pregnancy loss, prematurity, pre-eclampsia, intra-uterine growth restriction (IUGR) and maternal death. Univariate and multivariable logistic regression with generalised estimating equation (GEE) analyses were performed to determine the factors associated with APOs.

    RESULTS: A total of 153 patients with 240 pregnancies were included and the majority of the patients were Malay (69.9%), followed by Chinese (24.2%) and Indian (5.9%). The prevalence of APOs was 61.7% with the commonest complication being prematurity (28.3%), followed by pregnancy loss (24.6%) and pre-eclampsia (21.8%). Logistic regression model-based GEE analysis revealed that the independent predictors of APOs were active haematological system during pregnancy, pre-pregnancy active disease, Indian patients and positive lupus anticoagulant. Hydroxychloroquine use was associated with lower APOs including pre-eclampsia, prematurity and IUGR in the univariate analyses but it was no longer significant in the GEE analysis.

    CONCLUSION: The prevalence of APOs was high particularly among the Indian patients. Positive lupus anticoagulant and pre-pregnancy active disease were the factors strongly associated with APOs in our multi-ethnic cohort. Hydroxychloroquine may protect against APOs but further larger studies are needed to confirm this.

  20. Abd Talib AKA, Tan SC, Jamal R, Azizan EA, Shaharir SS, Abdul Murad NA
    Med J Malaysia, 2021 07;76(4):541-550.
    PMID: 34305116
    INTRODUCTION: Systemic lupus erythematosus (SLE) is a chronic and life-threatening autoimmune disease. Its prevalence and clinical manifestations are known to be particularly severe in the Asian populations. Although genetics is known to play an important role in SLE susceptibility and clinical manifestations, the specific polymorphisms associated with these phenotypes in Asia are unclear. Therefore, we aim to review the association of SLE genetic polymorphisms with lupus manifestations across Asian populations and their role in the pathogenesis of SLE.

    METHODS: A systematic search was conducted on PubMed, EBSCOHost, and Web of Science. We identified 22 casecontrol studies that matched our inclusion and exclusion criteria. Information such as study characteristics, genetic polymorphisms associated with SLE, and organ manifestations was extracted and reported in this review.

    RESULTS: In total, 30 polymorphisms in 16 genes were found to be associated with SLE among Asians. All included polymorphisms also reported associations with various SLE clinical features. The association of rs1234315 in TNFSF4 linking to SLE susceptibility (P=4.17x10-17 OR=1.45 95% CI=1.34-1.59) and musculoskeletal manifestation (P=3.35x10-9, OR=1.37, 95%CI= 1.23-1.51) might be the most potential biomarkers to differentiate SLE between Asian and other populations. In fact, these associated genetic variants were found in loci that were implicated in immune systems, signal transduction, gene expression that play important roles in SLE pathogenesis.

    DISCUSSIONS AND CONCLUSIONS: This review summarized the potential correlation between 30 genetic polymorphisms associated with SLE and its clinical manifestations among Asians. More efforts in dissecting the functional implications and linkage disequilibrium of associated variants may be required to validate these findings.

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