Idiopathic pulmonary fibrosis in Saudi Arabia: Demographic, clinical, and survival data from two tertiary care hospitals

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is rare and can be challenging to diagnose. Limited data is available from the Middle Eastern region, especially Saudi Arabia. METHODS: This was a retrospective study that looked at all the patients diagnosed with IPF between 2007 and 2012 at two tertiary care hospitals in Saudi Arabia. We collected the demographical, clinical, laboratory and radiological data from the patients’ medical records. Medications administered and 1 year survival was also assessed. RESULTS: Between 2007and 2012, 134 IPF patients were identified. Their baseline characteristics (Mean ± SD) included: age 64 ± 13 years, body mass index 29 ± 8 kg/m(2), FEV(1) 56 ± 15 percent of predicted, FVC 53 ± 13 percent of predicted, FEV(1)/FVC 0.81 ± 0.09, total lung capacity 75 ± 13 percent of predicted, diffusing capacity of the lung for carbon monoxide 57 ± 15 percent of predicted, on home oxygen at presentation 71 (53%), mean ejection fraction 0.50 ± 0.07, mean pulmonary artery systolic pressure (via echocardiogram) 40 + 22 mmHg, presentation mean S(pO2)92 ± 7%, presentation 6-min walk distance 338 ± 64 m and lowest S(pO2) during 6-min walk test 88 ± 5%. Patients were predominantly female (56%), and 42% of patients had diabetes and were active smokers. The IPF patients’ frequency of hospital admission (n = 99) was 2.4 ± 1.7 per year and duration of hospital stay (n = 99) was 17.4 ± 23.8 days. Overall 1 year survival in all IPF patients was good, 93% (124) patients remained alive after 1 year. CONCLUSIONS: In Saudi Arabia, IPF patients tended to be slightly older and the disease progression was somewhat slower than reported IPF cohorts in other populations. They had frequent hospital admissions and a long hospital length of stay. The influence of genetics and co-morbid diseases on the incidence and outcome of IPF should be explored further.