Idiopathic Pulmonary Fibrosis: Importance of Accurate Diagnosis and Treatment Fibrosis pulmonar idiopática: importancia de un diagnóstico preciso y tratamiento

In the last decade, Idiopathic Pulmonary Fibrosis (IPF), once a “mixed bag” where various interstitial diseases were included, has become a much better defined entity. This follows the expert statements on IPF, published in 20001 and 2011,2 which have established the diagnosis of this disease more precisely. In fact, in the 21st century, Non-Specific Interstitial Pneumonia (NSIP), Large Cell Interstitial Pneumonia (due to exposure to heavy metals), smoking-induced Pulmonary Fibrosis, and Chronic Hypersensitivity Pneumonitis (CHP), which present with clinical symptoms of IPF and a histological pattern of Usual Interstitial Pneumonia (UIP), are already found separate from the so-called Idiopathic Pulmonary Fibroses (PF) or individualized within them. However, in my opinion, the recommendations for the study of this entity described in the last international expert consensus on IPF in 20112 still do not place sufficient emphasis on recommending that the eventual cause of the PF be exhaustively sought; only if no etiology is found should we then call it idiopathic (IPF). Indeed, although it is recommended that an initial search be made for a possible cause of the symptoms, it is not stressed that this search must be painstakingly and repetitively continued at subsequent visits. The international consensus does not recommend the systematic determination of specific IgGs either, which is rightly recommended in Spanish Guidelines on the diagnosis and treatment of IPF3 published in this issue of Archivos de Bronconeumologia, and which has led to this Editorial. Moreover, due to their sensitivity and specificity, it would even be advisable to perform lymphocyte stimulation tests4 against antigens suspected of being the source of the fibrosis, in an attempt to systematically find a cause of CHP. Likewise, neither of the guidelines remark on the frequent etiological role of smoking in (Idiopathic) Pulmonary Fibrosis. In the 2011 international guidelines, the role of smoking is mentioned in the section on Risk Factors,2 and in the Spanish guidelines, in the section on Etiology and Risk Factors.3 It is obvious that in many patients with concomitant emphysema (paraseptal), some of whom also show a predominance of macrophages (pigmented) and