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Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital

Identifieur interne : 001744 ( Istex/Corpus ); précédent : 001743; suivant : 001745

Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital

Auteurs : C. L. Teh ; Y. C. Kuan ; J. S. Wong

Source :

RBID : ISTEX:FF650B669AEBE2C5680FBDF9FC06AF6ECDDC82C9

English descriptors

Abstract

Abstract: 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.

Url:
DOI: 10.1007/s00296-009-0938-z

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ISTEX:FF650B669AEBE2C5680FBDF9FC06AF6ECDDC82C9

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<Keyword>Pulmonary fibrosis</Keyword>
<Keyword>Pulmonary hypertension</Keyword>
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<title>Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital</title>
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<namePart type="given">L.</namePart>
<namePart type="family">Teh</namePart>
<affiliation>Unit of Rheumatology, Department of Medicine, Sarawak General Hospital, Jalan Hospital, 93450, Kuching, Sarawak, Malaysia</affiliation>
<affiliation>E-mail: tehchenglay@yahoo.com</affiliation>
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<namePart type="given">Y.</namePart>
<namePart type="given">C.</namePart>
<namePart type="family">Kuan</namePart>
<affiliation>Department of Medicine, Kuantan General Hospital, Kuantan, Pahang, Malaysia</affiliation>
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<name type="personal">
<namePart type="given">J.</namePart>
<namePart type="given">S.</namePart>
<namePart type="family">Wong</namePart>
<affiliation>Unit of Rheumatology, Department of Medicine, Sarawak General Hospital, Jalan Hospital, 93450, Kuching, Sarawak, Malaysia</affiliation>
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<abstract lang="en">Abstract: 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.</abstract>
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<genre>Keywords</genre>
<topic>Systemic sclerosis</topic>
<topic>Pulmonary fibrosis</topic>
<topic>Pulmonary hypertension</topic>
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<title>Rheumatology International</title>
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<title>Rheumatol Int</title>
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<dateIssued encoding="w3cdtf">2009-07-29</dateIssued>
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<genre>Medicine & Public Health</genre>
<topic>Rheumatology</topic>
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<identifier type="ISSN">0172-8172</identifier>
<identifier type="eISSN">1437-160X</identifier>
<identifier type="JournalID">296</identifier>
<identifier type="IssueArticleCount">24</identifier>
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<date>2009</date>
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<number>10</number>
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<start>1243</start>
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