The clinical profile, serological profile and management of patients diagnosed with systemic sclerosis in South Africa. A single center experience.

Abstract

Systemic sclerosis (SSc) is a complex and clinically heterogeneous disease with protean clinical manifestations, a chronic and frequently progressive course, and significant disability and mortality. The disease is characterized by fibrosis of the skin, internal organs and vasculopathy. While it can affect every organ, there is marked variability in patterns of skin and organ involvement, rates of disease progression, response to treatment and survival. The limited studies of SSc in South Africa (SA) have largely described the clinical manifestations and antibody profile in African patients. Since the last study from Durban in 1991 looking at the serological profile of SSc, there have been advances in serological and imaging investigations and therapeutics. Recent studies are predominantly from Johannesburg (JHB), and this study was conducted to look at the demographic profile, clinical characteristics and serological profile of patients with SSc in our local population. Inkosi Albert Luthuli Central Hospital (IALCH), a quaternary hospital, serves as a referral hospital for KwaZulu-Natal and part of the Eastern Cape. The Rheumatology Department provides outpatient and inpatient service for patients with SSc. Given the demography of KZN, the majority of patients attending the clinic are Indian and African Blacks subjects and this study brings the opportunity to document SSc in these populations. A retrospective chart review of the electronic records of all patients with SSc attending the rheumatology clinic IALCH was undertaken for the period January 2010 to December 2020. All patients with confirmed diagnosis of SSc and at least two visits to the clinic were included. Comparisons were made between limited and diffuse SSc and African and Indian patients using two-sided Fisher’s exact tests, Student’s t-tests and the Mann-Whitney U test for normally distributed and skewed numerical variables, respectively. This is the first study to report the differences in presentation of SSc between African and Indian patients in SA, which is reflective of the KZN population. African patients were significantly younger than Indian patients and more likely to have diffuse disease and a shorter disease duration. Interstitial lung disease and PAH, which are associated with poor outcomes, remain common manifestations, especially in diffuse disease. We confirm the absence of ACA in African patients; the mechanism of which requires further research.