Spectrum of disease and predictors of mortality in patients with idiopathic pulmonary fibrosis in South Africa

<bold>Aim:</bold> To assess baseline predictors of mortality in patients with idiopathic pulmonary fibrosis(IPF) at a tertiary hospital in KwaZulu-Natal, South Africa. <bold>Methods:</bold> A retrospective chart review of all patients with IPF who were treated from the year 2002 to 2019. <bold>Results:</bold> The study included 98 patients with IPF (27% Black patients;70% Asian Indian,58% female; median age 64 years). On presentation 45(46.4%) and 12(12.4%) patients had clinical signs of pulmonary hypertension and cor pulmonale respectively. The median forced vital capacity (FVC) was 1.59 (1.53-1.64) L. At 1 year follow-up, 20.8% had lost more than 100ml, and an additional 12.5% had lost more than 50ml. The median age of death was 66 (60-73) years. The median time to mortality from presentation was 15(6-35) months, while median time to mortality from symptom onset was 37(17-54) months. Significant risk factors for mortality included cough (HR 3.72; 95% CI 0.91-15.2;p= 0.07), home oxygen requirement(HR4.34; 95% CI 2.44-7.74;p= 0.001) , low FVC, low FVC % predicted, increased CT percentage lung involvement, Pulmonary artery(PA) diameter >3cm (HR 1.92; 95% CI 1.16-3.18;p= 0.01), PA/Aorta ratio >1(HR 1.7; 95% CI 1.01-2.84;p= 0.045),serum bicarbonate>29 mmol/L(HR 2.38; 95% CI 1.28-4.2;p= 0.005), serum sodium<135 mmol/L(HR 5.07; 95%CI 2.2-11.7;p=<0.001) and haemoglobin >16mg/dL(HR 2.96;95% CI 1.42-6.14;p= 0.004). <bold>Conclusion:</bold> Risk factors for mortality in the current study are in keeping with the rest of the world, although survival is worse. More studies are required in Africa to establish the true burden of disease and mortality on the continent.