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Systemic sclerosis in Sarawak: A profile of patients treated in the Sarawak General Hospital
Rheumatology International, 05/14/09

The CL et al. - Study demonstrates the rarity of systemic sclerosis in a multi-ethnic groups, predominantly Chinese, Sarawak natives and Malays with considerable lag time to diagnosis. Diffuse cutaneous systemic scleroderma was more common with rare pulmonary hypertension and renal involvement.

• A cross-sectional study of the demography, clinical and laboratory features of pts with SSc pts followed up from 1984 to 2007
• 23 cases; 96% were female; mean age of 50.3 yrs; mean disease duration of 6.02 yrs
• Pts comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%)

• Pts had a mean lag time to diagnosis of 24.8 mo
• All pts had sclerodermatous skin changes with 70% having diffuse scleroderma and 30% having limited scleroderma
• Common clinical manifestations 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%)
• 67% of pts had positive ANA with 33% positive Scl-70
• 87% received calcium channel blockers, aspirin (48%) and low-dose prednisolone (48%)
• 1 patient developed adenocarcinoma of the lung on f/u

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