Displaying all 17 publications

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  1. Fulltext A woman with pain and stiffness of hands
    Das Gupta E
    Malays Fam Physician, 2008;3(3):164-5.
    PMID: 25606146
    Matched MeSH terms: Scleroderma, Systemic*
  2. Fulltext Stiff hands and feet, facial deformities
    Thuraisingham C, Sinniah D
    J Fam Pract, 2016 Feb;65(2):121-4.
    PMID: 26977463
    The appearance of the skin on this woman's face, hands, and feet helped us to recognize an advanced case of an autoimmune disease.
    Matched MeSH terms: Scleroderma, Systemic*
  3. Pulmonary hypertension complicating systemic sclerosis: A successful pregnancy outcome
    Chew KT, Ahmad MF, Yong SL, Mohamed Ismail NA
    J Obstet Gynaecol, 2017 Jul;37(5):677-678.
    PMID: 28350528 DOI: 10.1080/01443615.2017.1284774
    Matched MeSH terms: Scleroderma, Systemic*
  4. Fulltext Demography, clinical and laboratory features of systemic sclerosis in a Malaysian rheumatology centre
    Pagalavan L, Ong SG
    Med J Malaysia, 2007 Jun;62(2):117-21.
    PMID: 18705442 MyJurnal
    A six year retrospective study of the demography, clinical and laboratory features of patients with systemic sclerosis (SSc) was carried out in Selayang Hospital. There were 61 cases seen between January 2000 and December 2005. Of these, 55 (90.2%) were females and 6 (9.8%) were males. Twenty-eight (45.9%) were Malays, 24 (39.3%) were Chinese and 9 (14.8%) were Indians. The mean age of onset was 38.8 years. Thirty-nine (64.0%) had limited cutaneous SSc, 21 (34.4%) had diffuse cutaneous SSc and one had localized morphoea. Raynaud's phenomenon was present in 82.6%, telangiectasia in 45.9%, calcinosis in 11.5%, sclerodactyly in 83.6%, digital pitting scars in 42.6%, digital infarcts/ulcers/gangrene in 23.0%, arthralgia/arthritis in 49.2% and gastroesophageal reflux disease (GERD) in 47.5%. Forty-three (70.5%) patients had interstitial lung disease. Seven patients had associated myositis, 7 systemic lupus erythematosus and 2 rheumatoid arthritis. Three had two other connective tissue diseases. Antinuclear antibodies were positive in 83.6% and anti-Scl 70 antibodies in 34.4%. This study demonstrates that limited cutaneous SSc is more common and there is a high incidence of interstitial lung disease in our population.
    Study site: Selayang Hospital, Kuala Lumpur, Malaysia
    Matched MeSH terms: Scleroderma, Systemic; Scleroderma, Systemic/complications*; Scleroderma, Systemic/diagnosis; Scleroderma, Systemic/immunology
  5. Fulltext Scleroderma secondary to silica exposure--a case report
    Anandan S, Othman M, Cheong I, Chin GL
    Singapore Med J, 1995 Oct;36(5):559-61.
    PMID: 8882549
    In recent years there have been many reports of connective tissue diseases especially scleroderma following exposure to silica and silicone. We report a 51-year-old Chinese who developed a scleroderma-like disease and pulmonary silicosis eight years after exposure to silica. To our knowledge, this is the first case to be reported in Malaysia.
    Matched MeSH terms: Scleroderma, Systemic/diagnosis; Scleroderma, Systemic/etiology*; Scleroderma, Systemic/physiopathology
  6. A systematic review of ultrasonography as an outcome measure of skin involvement in systemic sclerosis
    Ch'ng SS, Roddy J, Keen HI
    Int J Rheum Dis, 2013 Jun;16(3):264-72.
    PMID: 23981746 DOI: 10.1111/1756-185X.12106
    The modified Rodnan skin score is widely accepted as a validated tool to assess skin involvement in systemic sclerosis, which is a hallmark of this heterogeneous disease. Ultrasonography is increasingly being utilized in the study of other rheumatic diseases. The utility of ultrasonography to measure skin thickness in systemic sclerosis has been explored since three decades ago. The aim of this review was to examine the validity of ultrasonography as an outcome measure of skin involvement in systemic sclerosis. Original articles in English, published before December 2010, pertaining to the use of B mode ultrasound assessing skin involvement in systemic sclerosis were reviewed. Data were extracted with a focus on criterion and construct validity, reproducibility and responsiveness to change. Seventeen papers were analyzed. Skin thickness was most commonly studied, although skin echogenicity has also been examined. There was heterogeneity with regards to subjects, definitions used and sites imaged. Although there was limited information regarding reliability, when reported, the results showed excellent reproducibility. There was also a lack of construct and criterion validity and evidence for sensitivity to change. Ultrasound has potential as an outcome measure in systemic sclerosis. However, more work needs to be done in order to prove that it is a feasible outcome measure with proven validity.
    Matched MeSH terms: Scleroderma, Systemic/ultrasonography*
  7. The clinical relevance of sexual dysfunction in systemic sclerosis
    Bruni C, Raja J, Denton CP, Matucci-Cerinic M
    Autoimmun Rev, 2015 Dec;14(12):1111-5.
    PMID: 26235995 DOI: 10.1016/j.autrev.2015.07.016
    Systemic sclerosis is a chronic multi-organ autoimmune disease, leading to important clinical and psychological implications. Among organ complications, sexual dysfunction is a major issue for both male and female gender, with high prevalence and great impact on quality of life, although frequently not addressed by both clinicians and patients. While erectile dysfunction is the most common cause of sexual problems in males, genital tract and general physical changes are major contributors to sexual impairment in females. This review presents current state of the art on this topic, discussing published data on presentation, evaluation and therapeutic options.
    Matched MeSH terms: Scleroderma, Systemic/complications*
  8. Fulltext Systemic scleroderma with complete heart block
    Anuar M, Singham KT
    Med J Malaysia, 1983 Mar;38(1):65-7.
    PMID: 6633341
    Matched MeSH terms: Scleroderma, Systemic/complications*
  9. Co-existence of Marfan syndrome and systemic sclerosis: A case report and a hypothesis suggesting a common link
    Yap WF, Chong HC
    Int J Rheum Dis, 2020 Nov;23(11):1568-1573.
    PMID: 32969582 DOI: 10.1111/1756-185X.13965
    FBN1 gene encodes for the connective tissue protein fibrillin-1 which can also regulate the profibrotic cytokine transforming growth factor (TGF)-ß1. Mutations in the FBN1 gene cause Marfan syndrome (MFS), a genetic condition with defective connective tissues. FBN1 haplotypes and single nucleotide polymorphisms have also been reported to be associated with systemic sclerosis (SSc), a connective tissue disease characterized by fibrosis of multiple organs. Furthermore, the duplication of the Fbn1 gene causes a SSc-like disease in the TsK1 mouse model. To the best of our knowledge, there are no reports of MFS and SSc co-existing in a patient. Here, we describe a 46-year-old woman who presented with cardiac failure. She had a family history of MFS. Physical examination revealed marfanoid habitus and scleroderma features. Echocardiography demonstrated dilated cardiomyopathy with aortic root dilatation, aortic regurgitation and mitral regurgitation. Cardiac magnetic resonance imaging was consistent with dilated cardiomyopathy, mid-wall fibrosis at basal septal wall and dilated aortic root. Extractable nuclear antigen panel detected anti-Scl 70. She fulfilled Ghent criteria for MFS and satisfied American College of Rheumatology/ European League Against Rheumatism classification criteria for SSc. Although we do not have the FBN1 sequence in our patient, the co-existence of MFS and SSc in this patient raises the possibility of co-existence of distinct mutations in the FBN1 gene that could affect TGF-β signaling differently, resulting in divergent pathologic consequences - loss of structural integrity in MFS versus increased extracellular matrix deposition in SSc, and different clinical manifestations.
    Matched MeSH terms: Scleroderma, Systemic/complications*; Scleroderma, Systemic/diagnosis; Scleroderma, Systemic/genetics
  10. Fulltext Benign persistent pneumoperitoneum in systemic sclerosis
    Wang CL, Wang F, Wong KC, Jeyamalar R
    Singapore Med J, 1993 Dec;34(6):563-4.
    PMID: 8153727
    We describe a 50-year-old Chinese woman who had severe gastrointestinal manifestations from systemic sclerosis complicated by spontaneous pneumoperitoneum in the absence of either visceral perforation or pneumatosis cystoides intestinalis. This is a rare complication of systemic sclerosis; only four other cases have been reported. Recognition of this condition is important so as to avoid unnecessary surgery.
    Matched MeSH terms: Scleroderma, Systemic/complications*; Scleroderma, Systemic/pathology
  11. Rituximab for refractory digital infarcts and ulcers in systemic sclerosis
    Khor CG, Chen XL, Lin TS, Lu CH, Hsieh SC
    Clin Rheumatol, 2014 Jul;33(7):1019-20.
    PMID: 24722688 DOI: 10.1007/s10067-014-2579-1
    Systemic sclerosis is an uncommon connective tissue disease characterised by excessive deposition of collagen and vasculopathy which affects the skin and multiple internal organs. It is associated with autoimmunity, inflammation, vasculopathy and fibrosis. Managing Raynaud's phenonemon, digital infarcts and ulcers in systemic sclerosis patients is often a challenge particularly among rheumatologists. We report a case of systemic sclerosis patient with refractory digital infarcts and ulcers responded successfully with rituximab.
    Matched MeSH terms: Scleroderma, Systemic/complications; Scleroderma, Systemic/drug therapy*
  12. Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital
    Teh CL, Kuan YC, Wong JS
    Rheumatol Int, 2009 Aug;29(10):1243-5.
    PMID: 19373465 DOI: 10.1007/s00296-009-0938-z
    We performed a cross-sectional study of the demography, clinical and laboratory features of patients with systemic sclerosis patients followed up in our centre from 1984 to 2007. There were 23 cases with the majority of them (96%) being female. They have a mean age of 50.3 years and a mean disease duration of 6.02 (SD 5.82) years. Our patients comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%). They have a mean lag time to diagnosis of 24.8 (SD 34.8) months. All the patients have sclerodermatous skin changes with 16(70%) having diffuse scleroderma and 7(30%) having limited scleroderma. The common clinical manifestations found in our patients were Raynaud's phenomenon (91%), sclerodactyly (65%), digital ulcers (52%) and pulmonary fibrosis (52%). There was low incidence of pulmonary hypertension (13%) and renal involvement (4%). The majority of our patients (67%) have positive ANA with 33% positive Scl-70. The majority received calcium channel blockers (87%), aspirin (48%) and low-dose prednisolone (48%). One patient developed adenocarcinoma of the lung on follow-up. This study demonstrated the rarity of systemic sclerosis in our centre with considerable lag time to diagnosis in our patients. Diffuse cutaneous systemic scleroderma is more common in our centre with rare pulmonary hypertension and renal involvement.
    Matched MeSH terms: Scleroderma, Systemic/diagnosis*; Scleroderma, Systemic/pathology
  13. Clinical and autoantibody profile in systemic sclerosis: baseline characteristics from a West Malaysian cohort
    Sujau I, Ng CT, Sthaneshwar P, Sockalingam S, Cheah TE, Yahya F, et al.
    Int J Rheum Dis, 2015 May;18(4):459-65.
    PMID: 24618222 DOI: 10.1111/1756-185X.12322
    AIM: To evaluate the clinical and antibody profile of systemic sclerosis (SSc) in a Malaysian cohort.
    METHODS: Consecutive patients with SSc in University Malaya Medical Centre from March to November 2012 were included in this study. In addition to clinical characterization, all subjects underwent autoantibody testing using Euroline immunoblot assay. The association between clinical features and autoantibody profile was evaluated.
    RESULTS: There were 31, predominantly Chinese (45.2%), subjects. Limited cutaneous disease was the most common subtype (71%). Raynaud's phenomenon was the most commonly observed feature (83.9%). Nine (29%) had esophageal dysmotility symptoms and 23 (74.2%), including all patients with diffuse SSc, had symptoms of gastro-esophageal reflux disease (GERD). Restrictive pattern on pulmonary function test and evidence of lung fibrosis were seen in more than 70% of patients. Echocardiographic evidence of pulmonary arterial hypertension was seen in 58.1%. Telangiectasia, calcinosis, digital ulcers, digital pulp loss or pitting were seen more commonly in the diffuse subtype. The two most prevalent autoantibodies were anti-Scl-70 and anti-Ro-52. The presence of anti-Scl-70 was significantly associated with restrictive lung disease (P = 0.05). Anti-Ro-52 was associated with control subjects with other autoimmune diseases (P = 0.043). The presence of anti-PM-Scl-75 was associated with overlap syndrome (P = 0.032). Patients with anticentromere antibodies were more likely to have vasculitic rash (P = 0.012).
    CONCLUSION: In Malaysia, SSc most commonly affects the Chinese. Limited cutaneous is more common than diffuse subtype. Features of CREST (calcinosis, Reynaud disease, esophageal dysmotility, sclerodactyly, telangiectasia) are more commonly observed in the diffuse cutaneous subgroup. Anti-Scl-70 and anti-Ro-52 antibodies are promising biomarkers for pulmonary involvement in SSc.
    KEYWORDS: Malaysia; autoantibodies; clinical profile; systemic sclerosis
    Matched MeSH terms: Scleroderma, Systemic/ethnology; Scleroderma, Systemic/immunology*; Scleroderma, Systemic/physiopathology*
  14. Large fiber peripheral neuropathy in systemic sclerosis: A prospective study using clinical and electrophysiological definition
    Raja J, Balaikerisnan T, Ramanaidu LP, Goh KJ
    Int J Rheum Dis, 2021 Mar;24(3):347-354.
    PMID: 33432774 DOI: 10.1111/1756-185X.14042
    AIM: The reported prevalence of peripheral neuropathy in systemic sclerosis (SSc) is variable between 0.01% to 28%, probably due to differences in sample size, study design and population. Our aim is to determine the prevalence of large fiber peripheral neuropathy in SSc and to identify any contributing factors.

    METHOD: A prospective cross-sectional study of 60 SSc patients were evaluated for large fiber neuropathy using the modified clinical Total Neuropathy Score (cTNS) and nerve conduction study (NCS) of the upper and lower limbs. A combination of clinical (cTNS score ≥ 2) and NCS criteria (≥2 abnormal nerves including 1 sural [symmetrical polyneuropathy] and NCS abnormalities consistent with individual nerves/nerve roots [focal neuropathy]) was used to diagnose peripheral neuropathy.

    RESULTS: The majority had limited cutaneous subset (75%). Mean age was 55.73 (SD ± 13.04) years and mean disease duration was 8.61 (SD ± 8.09) years. Twenty-two (36.7%) had combined clinical and NCS criteria for peripheral neuropathy, 14 (23.3%) with symmetrical polyneuropathy and 8 (13.3%) with focal neuropathy. Symmetrical polyneuropathy patients had significantly lower hemoglobin levels (11.2 vs. 12.35 g/L; P = .047). Serum vitamin B12 levels were normal, therefore excluding vitamin B12 deficiency. No other associations were found for both polyneuropathy and focal neuropathy with demography, co-morbid diseases and SSc disease factors such as Raynaud's phenomenon and modified Rodnan skin score.

    CONCLUSION: Large fiber neuropathy is common in SSc patients, which could contribute to non-lethal burden in SSc with sensory loss and muscle weakness. Apart from lower hemoglobin in polyneuropathy, there were no associations with disease-specific features or co-morbid diseases.

    Matched MeSH terms: Scleroderma, Systemic/complications*; Scleroderma, Systemic/diagnosis; Scleroderma, Systemic/physiopathology
  15. Fulltext Sustained benefit from intravenous immunoglobulin therapy for gastrointestinal involvement in systemic sclerosis
    Raja J, Nihtyanova SI, Murray CD, Denton CP, Ong VH
    Rheumatology (Oxford), 2016 Jan;55(1):115-9.
    PMID: 26320139 DOI: 10.1093/rheumatology/kev318
    OBJECTIVE: IVIG is known to confer significant benefit in rheumatologic conditions, including inflammatory myopathy. This study aimed to assess the efficacy of IVIG across different aspects of internal organ involvement in refractory active SSc, particularly the gastrointestinal (GI) system.
    METHODS: SSc patients with overlap polymyositis who remained active and unresponsive to conventional disease-modifying agents and who subsequently received IVIG were identified. GI symptoms were assessed using validated questionnaires. The Medical Research Council Sum Score for muscle strength and modified Rodnan skin score (mRSS) were assessed. Serial measurements were undertaken at baseline prior to the first IVIG treatment and post-treatment in the most recent assessment.
    RESULTS: Fifteen SSc patients were consecutively recruited into this observational study. The mean duration of IVIG treatment was 2.3 years, with treatment frequency ranging from every 6 weeks to 4 months. Compared with baseline, there was a significant reduction in gastro-oesophageal reflux frequency and intensity mean scores (P = 0.006 and P = 0.013, respectively). Significant improvement in the Gastrointestinal Tract (GIT) 2.0 score from a baseline mean score of 1.07 (s.d. 0.67) to 0.60 (0.46) (P = 0.002) was observed. There was regression in the markers of muscle disease with a reduction in the mean (s.d.) Medical Research Council sum score and the median creatine kinase level (P = 0.001 and P = 0.025, respectively). Significant amelioration of the mean basal modified Rodnan skin score from 21.5 (s.d. 13.8) to 10 (10.6) (P = 0.005) was observed.
    CONCLUSION: IVIG may be a helpful adjunctive therapy in the amelioration of some key clinical aspects in refractory SSc. Sustained benefit from IVIG suggests a specific immunomodulatory effect on those with established SSc GI complications.
    Study site: Royal Free Hospital, United Kingdom
    Matched MeSH terms: Scleroderma, Systemic/complications; Scleroderma, Systemic/drug therapy*
  16. Recurrent spontaneous subdural hematoma secondary to immune thrombocytopenia in a patient with overlap syndrome
    Goh KG, Ong SG
    Lupus, 2015 Jan;24(1):90-3.
    PMID: 25305213 DOI: 10.1177/0961203314554248
    Patients with autoimmune connective tissue disease may manifest as overlap syndrome with features of systemic lupus erythematosus (SLE), systemic sclerosis, rheumatoid arthritis and myositis. Those presenting with active SLE can present with immune thrombocytopenia (IT) and may be complicated with subdural hematoma which, though rare, is potentially life-threatening. We report here a patient with overlap syndrome who had recurrent spontaneous subdural hematoma due to severe thrombocytopenia which did not respond to corticosteroids and azathioprine. Her platelet count became normal with three doses of low-dose intravenous cyclophosphamide (IV CYC) given at 3-weekly intervals. She remained in remission with maintenance therapy with azathioprine.
    Matched MeSH terms: Scleroderma, Systemic/complications
  17. High-resolution oesophageal manometry and 24-hour impedance-pH study in systemic sclerosis patients: association with clinical features, symptoms and severity
    Raja J, Ng CT, Sujau I, Chin KF, Sockalingam S
    Clin Exp Rheumatol, 2016 Sep-Oct;34 Suppl 100(5):115-121.
    PMID: 26843456
    OBJECTIVES: To evaluate the associations between objectively measured gastroesophageal involvement using high-resolution manometry and 24- hour impedance-pH study, and clinical presentations in systemic sclerosis (SSc) patients.
    METHODS: This cross-sectional study was conducted in University of Malaya Medical Centre (UMMC) with 31 consecutive SSc patients recruited into this study. Clinical symptoms of gastroesophageal involvement, high-resolution impedance-manometry and 24-hour impedance-pH monitoring were assessed. Their associations with serological features and other organ involvement were evaluated.
    RESULTS: Twenty-five (80.6%) patients had gastroesophageal reflux disease (GORD) symptoms, mainly heartburn (45.1%), regurgitation (32.2%) and dysphagia (29%). Using manometry, oesophageal dysmotility was detected in 24 (88.9%) patients, while hypotensive lower oesophageal sphincter (LOS) was observed in 17 (63%) patients. 21 (84%) patients had GORD based on pH study. Hypotensive LOS was significantly associated with presence of digital ulcers. The main gastroesophageal symptoms were absent in majority of the SSc patients including in those with severe gastroesophageal manifestations demonstrating failed peristalsis >75%, hypotensive LOS, Demeester score >200 and acid reflux >200 per day. Demeester score >200 is associated with severity of GORD symptoms. Demeester score >200 was also associated with restrictive lung pattern (p=0.001). Significant association between GORD severity (daily number of acid reflux episodes >200) and pulmonary fibrosis was seen (p=0.030).
    CONCLUSIONS: The presence and severity of gastroesophageal symptoms may not accurately reflect the seriousness of oesophageal involvement. GORD severity is associated with presence of restrictive lung pattern and pulmonary fibrosis. Oesophageal manometry and 24-hour pH study should be considered more frequently in the assessment of SSc patients.
    Matched MeSH terms: Scleroderma, Systemic
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