Editorial
Clinical Anti-Inflammatory & Anti-Allergy Drugs, 2014, Vol. 1, No. 2
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Editorial Idiopathic Pulmonary Fibrosis Idiopathic Pulmonary Fibrosis (IPF) is a type of chronic and escalating interstitial pneumonia whose main characteristic is scarring of lung tissue. IPF is rare and more prevalent in men. It usually presents at mean age of 66 and its prognosis is poor. Median survival time ranges from 2.5 to 5 years [1]. However, the clinical course of disease can change considerably from patient to patient. Some show slow progression over many years and others present acute exacerbations and rapid loss of lung function leading to early death. It has been previously suggested that varying rates of disease progression could be related to different IPF phenotypes. Patients with comorbidities present worse prognosis. The three presentations of IPF: rapidly progressive, IPF combined with emphysema and pulmonary fibrosis and IPF with pulmonary hypertension may represent distinct phenotypes [2-5]. Diagnosis depends on several factors such as clinical symptoms, images and lung biopsy. When the biopsy cannot be performed, diagnosis should be based on clinical guidelines. When highresolution computed tomography shows peripheral, sub-pleural reticular opacities in lower lobes together with sub-pleural honeycomb features, IPF is associated with usual interstitial pneumonia (UIP). IPF/UIP presents a normal lung pattern and spots of dense fibrosis [6]. As it has been previously described by “Antoniou KM et al.”; it is of utmost importance to take into consideration confounding diseases such as idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis), autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (such as occupational exposures) [7-9] for an accurate diagnosis. Several studies have shown that selected features such as clinical history, physical examination, radiographic images and biological biomarkers are related to a higher mortality risk. However, the combination of these predictors is not yet accepted as a standardized method to define prognosis or stage of disease [10]. Several efforts have been made to understand the mechanism involved in the development IPF. MeiLan K. and colleagues have found an association between progression of idiopathic pulmonary fibrosis and the lung microbiome and have demonstrated the presence of Streptococcus and Staphylococcus in these patients bronchoalveolar lavage [11]. In the future, these data could be used to support a new protocol to study the role of antibiotics as potential IPF treatment. There are new treatments available for certain IPF patients but they can only help to prevent disease progression, since prediction of response is not possible due to the heterogeneity of the condition. Major IPF causes are still poorly understood, though it is thought that it could result from repeated alveolar injury and abnormal repair. It is necessary to continue studying the triggers for injury to generate new potential therapeutic targets. This special issue aims to show different presentations of IPF and reinforce the need to continue investigating molecular pathways and clinical tools to improve therapeutic options for patients with IPF. REFERENCES [1] [2] [3] [4] [5] [6]
Ley B, Collard HR, King TE Jr, et al. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183: 431-40. Fell CD. Idiopathic pulmonary fibrosis: phenotypes and comorbidities. Clin Chest Med 2012; 33(1): 51-7. Selman M, Carrillo G, Estrada A, et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One 2007; 2: e482. Cottin V, Cordier JF. The syndrome of combined pulmonary fibrosis and emphysema. Chest 2009; 136: 1-2. Corte TJ, Wort SJ, Wells AU. Pulmonary hypertension in idiopathic pulmonary fibrosis: a review. Sarcoidosis Vasc Diffuse Lung Dis 2009; 26: 7-19. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788-24.
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[7] [8] [9] [10] [11]
Editorial
Crestani B. The respiratory system in connective tissue disorders. Allergy 2005; 60: 715-34. Antoniou KM, Margaritopoulos G, Economidou F, Siafakas NM. Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement. Eur Respir J 2009; 33(4): 882-96. Maher TM, Wells AU, Laurent GJ. Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms? Eur Respir J 2007; 30(5): 835-9. Kolb M, Collard HR. Staging of idiopathic pulmonary fibrosis: past, present and future. Eur Respir Rev 2014; 23(132): 220-4. Han MK, Zhou Y, Murray S, et al. Lung microbiome and disease progression in idiopathic pulmonary fibrosis: an analysis of the COMET study. Lancet Respir Med 2014; 2(7): 548-56.
Dra Glenda Ernst Guest Editor Research Coordinator, Respiratory Medicine British Hospital Perdriel 74, C1280AEB Buenos Aires Argentina Tel/Fax: +54-11-43096400 E-mail:
[email protected]
