Clinical profile of scleroderma associated interstitial lung disease at a tertiary hospital in KwaZulu-Natal, South Africa: a retrospective 7 years review.

Abstract

Background: Interstitial lung disease (ILD) is one of the most serious complications among patients with scleroderma. It is associated with significant morbidity and mortality. Little is known about the epidemiology of scleroderma-associated interstitial lung disease in sub-Saharan Africa. Thus, we aimed to determine the prevalence, clinical characteristics and outcomes of patients with scleroderma-ILD. Methods: A retrospective electronic chart review was conducted of patients with systemic sclerosis seen between January 2010 and December 2016 in the Departments of Pulmonology and Rheumatology at Inkosi Albert Luthuli Central Hospital, Durban, South Africa. Results: A total of 146 patients with systemic sclerosis (SSc) were seen during the study period. Fifty-five patients had systemic sclerosis-associated ILD, giving a prevalence of 37.7%. The median age was 51 (IQR 41-60) years, 87% of patients were female and 56% were of Indian descent. Dyspnoea was the presenting complaint in 47 (85.4%) patients, while 16 (29.1%) presented with cough. Antinuclear factor (ANA) was positive in 50 patients (90.9%), anti-Scl-70 antibodies were positive in 21 (38.2%). Thirty-seven patients (67.3%) received immunosuppressants, with 27 (49.1%) receiving cyclophosphamide as induction therapy, while 18 (32.7%) patients did not receive any specific therapy. Most patient symptoms remained static during the period of observation. Usual interstitial pneumonia (UIP) pattern was the most common radiological diagnosis. Follow-up computer tomography (CT) scans were available in 48 (87%) patients, with the majority of patients showing no significant radiological changes between their first and last CT scans. Follow-up lung function testing showed a statistically significant decrease in median forced vital capacity (FVC) of 0.09l (p=0.011). Overall 20 (36.4%) patients had a significant decline in FVC, while 7 (12.7%) had an improvement in FVC. Conclusion: ILD is common in systemic sclerosis, affecting 1 in 3 patients with systemic sclerosis in this cohort. Immunosuppressant treatment may arrest or retard the rate of decline in lung function.