Idiopathic Pulmonary Fibrosis (IPF) incidence and prevalence in Italy

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Nera Agabiti
Maria Assunta Porretta
Lisa Bauleo
Angelo Coppola
Gianluigi Sergiacomi
Armando Fusco
Francesco Cavalli
Maria Cristina Zappa
Rossana Vignarola
Stefano Carlone
Gianpiero Facchini
Salvatore Mariotta
Paolo Palange
Salvatore Valente
Giovanna Pasciuto
Gabriella Pezzuto
Augusto Orlandi
Danilo Fusco
Marina Davoli
Cesare Saltini
Ermanno Puxeddu


Idiopathic pulmonary fibrosis, epidemiology, ICD9-CM


Background: Studies of Idiopathic Pulmonary Fibrosis (IPF) epidemiology show regional variations of incidence and prevalence; no epidemiological studies have been carried out in Italy. Objective: To determine incidence and prevalence rates of IPF in the population of a large Italian region. Methods: in this cross-sectional study study data were collected on all patients of 18 years of age and older admitted as primary or secondary idiopathic fibrosing alveolitis (ICD9-CM 516.3) to Lazio hospitals, from 1/1/2005 to 31/12/2009, using regional hospital discharge, population and cause of death databases. Reporting accuracy was assessed on a random sample of hospital charts carrying the ICD9-CM 516.3, 516.8, 516.9 and 515 codes, by reviewing radiology and pathology findings to define cases as IPF “confident”, “possible” or “inconsistent”. Results: Annual prevalence and incidence of IPF were estimated at 25.6 per 100,000 and 7.5 per 100,000 using the ICD9-CM code 516.3 without chart audit while they were estimated at 31.6 per 100,000 and at 9,3 per 100,000 for the IPF “confident” definition after hospital chart audit. Conclusion: The data provide a first estimate of IPF incidence in Italy and indicate that incidence and prevalence in southern European regions may be similar to those observed in northern Europe and North America.
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