Combined Pulmonary Fibrosis and Emphysema (CPFE)

Combined pulmonary Fibrosis and Emphysema (CPFE)Ong Wei Jun DanThe Causes, Consequences and Differences Between Pulmonary Fibrosis or Emphysema AloneAbstractCombined pneumonic fibrosis and pulmonic pulmonic pulmonary emphysema (CPFE) is a multiform disease and untreated disease which consists of two diseases. It is difficult for respiratory therapists or respiratory physicians to differentiate amongst CPFE versus idiopathic pulmonic fibrosis (IPF)/emphysema alone(predicate). There is an increased recognition of the coexistence of emphysema and pulmonary fibrosis in undivideds. The experience of two diseases outcomes in chronic dyspnea, speed-lobe emphysema and lower lobe fibrosis, and severely change magnitude diffusion of gas exchange with preserved lung volumes. CPFE is also frequently complicated by pulmonary hypertension, lung disgrace and even lung cancer. This causes CPFE tolerants to feel sire a low quality of life and a low 10-year survival rate. Currently, i n that respect be no known sermons for CPFE long-sufferings with the exception of lung transplantation. Thus, clinical evaluations are necessary to differentiate between chronic obstructive pulmonary disease (COPD) and pulmonary fibrosis, and to recognize that CPFE is a incomparable entity by looking at the expiration in radiological, pathological and metamorphosis features in order to find give way treatment for CPFE.IntroductionAbout 11 million Americans stick out inveterate Pulmonary Obstructive Disease (COPD) and out of these, most are described with pulmonary emphysema. The etiology of emphysema found that 80% of casefuls are caused by butt end sess, which causes alveolar consonant membranes to break down, creating huge alveoli (called blebs) that littleer get up area and weaker groynes than normal alveoli. This causes the low perfusion of atomic number 8 due(p) to decrease in surface area. In addition, approximately 50,000 new cases of Idiopathic Pulmonary Fibrosis (IPF) are diagnosed each year. IPF is a restrictive respiratory disease, and it is the most common of the idiopathic lung diseases. IPF causes thickening of the alveolar capillary tubing membrane, which results in minimal gas exchange between the alveolar and the blood capillaries. Both diseases lead to the decreased efficacy of type O delivery.CPFE is a combination of both IPF and emphysema. However, it is usually treated as IPF and unattended or excluded in the diagnosis of emphysema. COPD and pulmonary fibrosis halt different pathologies, metabolic pathways and radiological characteristics, and were on that pointfore regarded as separate entities for a very great time. However, in recent years, there is about recognition of the coexistence of pulmonary fibrosis and emphysema in patients. As such, it is very important to know the differences between CPFE versus emphysema or pulmonary fibrosis alone in order to find a treatment or prevent the patients see to its fr om further deteriorating.In the following years, studies had shown that CPFE patients have a coincidental incident of early emphysema and at later epoch of IPF, especially for smokers with many tamp years.1 However, in recent studies there is a correlation between the occurrence of the combination between lower lobe pulmonary fibrosis and upper lobe emphysema. These two diseases have been observed coexisting in great frequencies which are therefore called combined pulmonary fibrosis and emphysema (CPFE) and there is a need to distinguish them as distinct entities. There are some studies taking place to better understand the pathophysiology of the condition and find the viable causes of CPFE such as genetic actors or any biological metabolism pathways which whitethorn encourage its training. CPFE is normally caused by heavy smoking, exercise hypoxemia, upper lobe emphysema and lower lobe pulmonary fibrosis, unexpected lung volume and severe decline of carbon monoxide trans fer.2Whether the combination of both emphysema and pulmonary fibrosis is a unique clinical entity still remains unknown. For some of the population in the health check community, it is a coincidental occurrence of two smoking-related diseases on one person, versus the coexistence of the standardizedities of COPD and lung cancer. However, many different studies have shown and suggested that interstitial lung unnaturalities, which are normally caused by IPF, have are inversely related to emphysema in smokers. In fact, base on the actors assistant X-Ray images, most patients who have many drove years with IPF do not have any signs of having emphysema. Similarly, most patients who have emphysema do not have any signs of IPF in their agency X-Ray. Hence, the combination of both pulmonary fibrosis and emphysema may be a direct result of heavy smoking or many pack years which reflects the uniqueness in individual susceptibilities.Even though medical examination professionals move to use chest X-Rays for any respiratory disoblige, as it is chintzy and considered a fast diagnostic tool, it is unable to properly diagnose the CPFE syndrome. other alternative would be to use High-Resolution Chest Computed Tomography (HRCT), which is the precisely tool to diagnose the syndrome. The CPFE syndrome consists of heterogeneous syndromes, in which syndromes differ from one individual to another and resulting in no actual definition of the syndrome for CPFE. This makes it difficult to diagnose CPFE with the current pulmonary function test, as CPFE patient results look identical to those of patients diagnosed with pneumonia. From past research and observations, CPFE is frequently complicated with pulmonary hypertension, acute lung injury and the possibility of lung cancer, resulting in very poor prognoses. Treatments for CPFE patients with severe pulmonary hypertension have not been found and have largely proven otiose in curing the disease apart from a wholesale lun g transplant.The acknowledgment of patients with CPFE is needed due to the uniqueness and complication of the diseases history. Since CPFE has not yet attracted the charge of researchers and healthcare practitioners, there have not been many studies focused on finding the differences between pulmonary fibrosis, emphysema and CPFE. Currently, there is no coherent way to differentiate the factors, signs and syndromes when diagnosing CPFE patients from other obstructive respiratory diseases. This has resulted in many medical practitioners failing to immediately recognize CPFE in patients diagnoses. commonwealth distribution of Emphysema, IPF and CPFEThe prevalence of the disease emphysema was reported to be at about 24.5 per 1,000 in America, while the prevalence of IPF varied from 14 to 42.7 cases per 100,000. Therefore, emphysema is a much(prenominal) common disease as compared to IPF. However, there are no studies that account for the prevalence of CPFE. Some of the reported ob servations show that the coincidence of patients with CPFE detected on HRCT scans range from 8% to 51% in IPF patients. On the other hand, the proportion of pulmonary fibrosis found in patients with emphysema is less than 10% using the HRCT. This variation of proportion of prevalence in CPFE may be due to the different types and complications arising from the diagnosis of emphysema when evaluated by chest X-Ray and HRCT.Patients with CPFE tend to be older men who tend to have many pack years of smoking. Previous studies have shown that there is no significant difference when varying the number of pack years against the occurrence of COPD such as emphysema and CPFE. However, patients with CPFE and those with COPD usually have a long history of smoking as compared to patients with IPF. Many studies have reported that masculine have higher prevalence then fe manly in having respiratory disease syndrome, and could be due to men tending to have more pack years as compared to females. It may also be due to the genes of men which predispose them to succumbing to COPD or CPFE. Even though both IPF and emphysema have proven to be more common in male smokers than female smokers, it does not necessarily mean that gender plays an important endangerment factor in the contraction of CPFE. More studies are needed to take root how gender differences affect this syndrome.Pathology pathway of CPFETill now, there are no conclusive findings for pathogenies of CPFE. There are no take a crap conclusions on the development of CPFE, whether emphysema and or pulmonary fibrosis progress independently or whether there are synergistic qualities between the two. There may be some mechanisms involving cytokines, beta receptors or signaling pathways which have not been discovered. Thus, both pulmonary fibrosis and emphysema may tend to occur in genetic might individuals with from exposure to environmental factors such as smoking or occupational hazard and chemicals.Case Study of a CP FE patient (Occupational exposure)A case study journal report on a male patient aged 73 years old in 2015 gives one of the more detailed analysis of Microscopic Polyangiitis (MPA), a disease that precedes by CPFE. The patient worked as a metalworker and had 25 pack years. He was admitted to the infirmary due to progressive dry coughing and he was later diagnosed with CPFE. He eventually died due to complications from CPFE, which resulted in severe pneumococcal pneumonia with acute lung injury. His arterial blood gas result was normal with a fairly abnormal range in his pulmonary function test (PFT). There were clear signs of emphysema and IPF from his CT scan and Chest X-Ray (Kyoko Gocho, 2015). MPA is a general necrotizing vasculitis of small vessels associated with numerous types of antibodies in particular myeloperoxidase- antineutrophil cytoplasmic antibody (MPO-ANCA). Oxidation induce by MPO-ANCA may trigger pulmonary fibrosis due to alveolar hemorrhage, resulting in pulmonary capillaritis (an inflammation of pulmonary capillary). This causes pulmonary fibrosis as the alveolar capillary wall thickens (Kagiyama, 2015)Correlation of smoking with CPFE patientsA common etiology factor for CPFE is smoking. Tobacco smoke contains 4000 chemical substances, including Kaolinite or aluminum silicate, an organic industrial material. Studies show that inhalation of this organic industrial substance go away result in hyperactive macrophages, which in turn will lead to respiratory bronchiolitis and emphysema (King, 2005). Currently, there are no studies for the association of tobacco smoking resulting in IPF, other factors such as environmental factors in genetically-predisposition individuals may play a key role in resulting IPF. The association between CPFE and lung cancer may reflect the susceptibility linked to long marge smoking which causes chronic smoking-induced inflammation. These were done on several other studies on the relationship between emphysemaand I PF.3,4Pathological findings (Diagnostic Imaging)Patients who have acute respiratory distress syndrome such as COPD, pulmonary fibrosis or even CPFE, will tend to have more difficulty breathing due to the use of helper muscles and the need to constantly supply supplemental oxygen to meet the oxygen level demanded by the body. For some of the patients, a high flow of oxygen is required (flow rate of more than 60L/min) to meet their inspiratory demand. Patients with CPFE have a confused and undetermined ventilation/ perfusion ratio due to emphysema make low perfusion and IPF having low ventilation. This results in both ventilation of oxygen to the alveoli and perfusion of capillaries to be diminished, leading to dead space and transfer. Emphysema results in the reduction of alveoli-capillary surface membrane by forming a bleb that causes air-trapping, whereas pulmonary fibrosis scars the alveolis tissue, creating a shunt that causes ventilation of the oxygen to the alveoli to be ine fficient, resulting in the patients body tissue being unable to get a sufficient amount of oxygen.Other unprecedented syndromes found in COPD patients are chronic cough and sputum output in volume greater than one shot full frosting due to inflammation of bronchi and impairment of the mucociliary clearance, presumably due to the effects of smoking. Patients with IPF may show progressive shortness of breath, loud expiratory wheezing sounds and if the condition is worse cyanosis may appear on the patient. CPFE from previous clinical studies shows that it is similar to IPF. On close physical examination, by doing chest auscultation, it was found that more than 80% of CPFE patients will emit inspiratory dry crackles sounds due to the underlie pulmonary fibrosis. About 40 to 50% will have soma clubbing and poor capillary refill.As of now, there is no arranged definition for CPFE. However, it is very important to diagnose it early. Diagnostic criteria for CPFE include radiologica l findings by using either chest X-Ray or HRCT these images will appear as upper-lobe emphysema with fibrosis like blebs, lower-lobe honeycombing with subpleural reticular opacities, thick wall cystic lesions, and sometimes ground glass opacities.2Table 1 comparing of clinical characteristics difference between CPFE, emphysema and IPF patients group (measures of Framingham variables)CPFEIPFEmphysemap-value experiment size22817Age (in years)Median73.574780.7Range59-9656-8948-86Number of pack yearsMedian6443750.64Range20-5030-8015-65Table 2 Comparison of clinical characteristics difference between CPFE, emphysema and IPF patients group (Pulmonary Function Test)CPFEIPFEmphysemap-value alert capacity2.520.722.340.862.850.610.52Vital capcity (%)83.122.168.027.787.012.40.29FEV12.010.191.600.241.570.220.28FEV1/FVC(%)76.83.3181.84.4555.64.06 70%, this results being emphysema to be ignored or overlooked. Physician, healthcare workers and respiratory therapists should be aware of its existe nce. More autopsies should be recognized such as thick-walled cystic lesion and idiopathic interstitial pneumonia should be recognized as both of these can be found in CPFE patients but are seldom found in emphysema/IPF alone patients. A deeper understanding of the pathophysiology is needed for CPFE and the factors that causes the syndrome of CPFE should be explored further with more clinical studies so as to develop effective treatments or therapeutic strategies for CPFE patients.References Hiwatari H., S. S. (1993). Pulmonary emphysema followed by pulmonary fibrosis of undetermined cause. Respiration, 60(6).Cottin V., H. N. (2005). Combined pulmonary fibrosis and emphysema a distinct underrecognised entity. European respiratory Journal, 26(4).Kaplan R. M. (2015). Quality of Well-being Outcomes in the National Emphysema Treatment Trial. Chest Journal, 147(2).Kagiyama C., N. T. (2015). Antineutrophil cytoplasmic antibody-positive conversion and microscopic polyangiitis development in patients with idiopathic pulmonary fibrosis. BMJ Open Respiratory Research, 2(1).Inomata M., A. M. (2013). An autopsy study of combined pulmonary fibrosis and emphysema correlations among clinical, radiological, and pathological features. BMC Pulmonary Medicine, 104(14).King, C. G. (2005). COPD a dust-induced disease? Chest Journal, 128(4).Kyoko G. (2015). Microscopic polyangiitis preceded by combined pulmonary fibrosis and emphysema. Respiratory Medicine Case Reports, 10(2).Papaioannou A. I., E. A. (2016). Combined pulmonary fibrosis and emphysema The many aspects of a cohabitation contract. Respiratory Medicine, 117(10).9. Portill K., J. M. (2011). Combined Pulmonary Fibrosis and Emphysema Syndrome A New Phenotype deep down the Spectrum of Smoking-Related Interstitial Lung Disease. Pulmonary Medicine , 2012(1).