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Clinical and laboratory manifestations of systemic sclerosis (scleroderma) in Black South Africans
1Department of Medicine, Chris Hani Baragwanath Hospital, Johannesburg, South Africa.
Insights
Systemic sclerosis (SSc) in South African Blacks shows early onset and a high prevalence of diffuse cutaneous SSc (dcSSc) and myositis. Findings resemble those in African-Americans, suggesting shared genetic or environmental factors.
Area of Science:
- Rheumatology
- Immunology
- Epidemiology
Background:
- Systemic sclerosis (SSc) is a complex autoimmune disease with varying clinical presentations.
- Understanding ethnic and geographic variations in SSc is crucial for targeted healthcare and research.
Purpose of the Study:
- To describe the clinical characteristics and outcomes of Black patients with SSc in South Africa.
- To compare these findings with previously reported data, particularly in African-Americans.
Main Methods:
- Retrospective study of 63 Black patients with SSc at a tertiary hospital.
- Data collection included demographics, clinical subset (dcSSc, lcSSc), comorbidities, and serological markers.
- Analysis of disease subset, sex, and potential environmental exposures (gold mining).
Main Results:
- Mean age of SSc onset was 36.1 years, with a female:male ratio of 4.6:1.
- Diffuse cutaneous SSc (dcSSc) was the predominant subset (41 patients).
- Pulmonary fibrosis (56%), myositis (37%), and antinuclear antibody (ANA) positivity (98%) were common; anti-centromere antibodies were absent. Myositis and reduced lung function were more frequent in dcSSc.
- Arthralgia/arthritis more common in women; calcinosis more frequent in men.
Conclusions:
- SSc in this South African Black population exhibits early onset, a high proportion of dcSSc, and inflammatory features, aligning with findings in African-Americans.
- The absence of anti-centromere antibodies is a notable characteristic.
- Environmental factors related to gold mining may play a role in disease presentation.
Abstract:
A retrospective study of systemic sclerosis (SSc) in Blacks attending a tertiary hospital on the Witwatersrand, South Africa, was undertaken. The female:male ratio of the 63 patients was 4.6:1 and the mean age of onset of SSc was 36.1 yr. Four of the 11 males were ex-goldminers and nine females resided close to goldmines. Forty-one patients had diffuse cutaneous SSc (dcSSc), 18 had limited cutaneous SSc (lcSSc) and four were unclassified. Overall, 56% had pulmonary fibrosis, 37% had myositis and 98% were antinuclear antibody (ANA) positive, with a notable absence of anti-centromere antibodies. Subset comparisons showed myositis and a reduced forced vital capacity to be significantly more common with dcSSc than lcSSc. The only significant sex differences were that arthralgia/arthritis was more common in women, while calcinosis occurred more frequently in men. Seven of the eight known deaths occurred in patients with dcSSc. These findings, particularly the age of disease onset, predominance of the dcSSc subset, inflammatory features of myositis and a raised erythrocyte sedimentation rate, and absence of anti-centromere antibodies, are similar to those reported previously in African-Americans.