Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Oct 22:37:176.
doi: 10.11604/pamj.2020.37.176.22557. eCollection 2020.

Systemic sclerosis in sub-Saharan Africa: a systematic review

Affiliations

Systemic sclerosis in sub-Saharan Africa: a systematic review

Julian Nicolas Erzer et al. Pan Afr Med J. .

Abstract

Systematic studies on connective tissue disorders are scarce in sub-Saharan Africa. Our aim was to analyse the published clinical data on systemic sclerosis (SSc) in sub-Saharan Africa. A systematic review was carried out in accordance with the PRISMA guidelines. We screened the Embase, PubMed and African Health Sciences databases for literature published until March 2018. Searches produced 1210 publications. After abstract and full-text screenings, 91 publications were analysed, and epidemiological information and clinical features extracted. Publications were mostly publications case reports (36%), cross-sectional studies (26%) and case series (23%) and came predominantly from South Africa (45%), Nigeria (15%) and Senegal (14%). A total of 1884 patients were reported, 66% of patients came from South Africa. The patients were between 4 and 77 years old; 83% of patients were female. Overall, 72% had diffuse SSc. Raynaud´s phenomenon was reported in 78% and skin ulcerations in 42% of patients. Focal skin hypopigmentation was common and telangiectasia not frequent. Interstitial lung involvement was reported in 50%, pulmonary hypertension in 30%, heart involvement in 28% of patients. Oesophageal reflux was observed in 70% and dysphagia in 37% of patients. Antinuclear antibodies were positive in 65% of patients. Anti-centromere autoantibodies (9.2%) and RNA polymerase 3 antibodies (7.1%) were rare and anti-fibrillarin most frequent (16.5%). SSc presentations in sub-Saharan Africa differ from those reported in Europe and America by a frequent diffuse skin involvement, focal skin hypopigmentation and a high prevalence of anti-fibrillarin autoantibodies.

Keywords: Systemic sclerosis; connective tissue disease; sub-Saharan Africa; systematic review.

PubMed Disclaimer

Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
flow chart of paper collection and selection
Figure 2
Figure 2
origin of publications and patients

References

    1. Tyndall A, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR scleroderma trials and research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809–1815. - PubMed
    1. Steen V, Domsic RT, Lucas M, Fertig N, Medsger TA. A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis. Arthritis Rheum. 2012;64(9):2986–2994. - PMC - PubMed
    1. Goetz RH, Berne CB. The pathology of progressive systemic sclerosis (generalized scleroderma) with special reference to changes in the viscera. Clin Proc (Cape Town) 1945;4:337–392.
    1. Erasmus LD. Scleroderma in goldminers on the Witwatersrand with particular reference to pulmonary manifestations. S Afr J Lab Clin Med. 1957;3(3):209–231. - PubMed
    1. Tikly M, Ickinger C, Dire Z. Causes and predictors of death in South Africans with systemic sclerosis. J Scleroderma Relat Disord. 2018;3:113–154.

Publication types