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Editorial article
Bronchiectasis: The disease that was never orphan
Bronquiectasias: la enfermedad que nunca fue huérfana
Miguel Ángel Martínez-García
Servicio de Neumología, Hospital Universitario y Politécnico La Fe, Valencia, Spain
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    "titulo" => "Bronchiectasis&#58; The disease that was never orphan"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Bronchiectasis&#44; understood as dilatation of the bronchial lumen&#44; was first described in the early 19th century by La&#235;nnec&#46; This disease has claimed millions of lives ever since&#44; especially in times of pandemics generated by microorganisms that cause severe pulmonary damage&#44; such as tuberculosis&#46; Luckily&#44; the number of diagnoses and deaths related to this disease decreased dramatically with the surge of preventive medicine and antibiotic therapy during the 20th century&#46; However&#44; far from becoming extinct&#44; bronchiectasis has continued to be present to date&#44; albeit with other forms of radiological presentation that could not be diagnosed without the appropriate imaging methods that are currently available&#46; It is now a well-known fact that this entity can impact the patients&#8217; prognosis and the evolution of the underlying disease that caused it&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">This became evident when&#44; at the beginning of the 1980s of the last century&#44; a very significant increase was observed in the number of diagnoses of bronchiectasis of varying etiologies&#46; These included&#44; on the one hand&#44; the omnipresent tuberculosis and the emergence of the pandemic of human immunodeficiency virus &#40;HIV&#41; infections&#46; On the other&#44; the increasing longevity of the population&#44; which allowed bronchiectasis-generating diseases to be able to cause this entity before the patients&#8217; death&#46; And&#44; finally&#44; the increasing variety of immunosuppressive drugs that were highly effective for the treatment of certain diseases&#44; but&#44; in turn&#44; favored pulmonary infections&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">However&#44; there were two predominant causes of this increase in the number of diagnoses of bronchiectasis&#46; In the first place&#44; the awareness of the existence of this entity following Cole&#39;s description of its causative pathophysiological mechanism &#40;Cole&#39;s vicious circle&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> On the second hand&#44; and of particular relevance&#44; the massive use of thoracic computed tomography &#40;particularly high-resolution computed tomography &#91;HRCT&#93;&#44; which is the gold standard in the diagnosis of this condition&#41;&#44; which allowed clinicians to examine the pulmonary parenchyma and airways with unprecedented accuracy&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">As a result of the significant increase in the number of diagnoses of this condition and the certainty that it was associated with a worse prognosis in both patients and their underlying diseases&#44; various national<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;7</span></a> and international<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> patient registries<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> and clinical practice guidelines began to be developed&#46; The main objective of these tools was to gather more scientific evidence on this disease and offer therapeutic recommendations&#44; given that&#44; thus far&#44; treatment of bronchiectasis&#44; in the best of cases&#44; was extrapolated from that of other airway diseases&#44; such as chronic obstructive pulmonary disease &#40;COPD&#41; or asthma&#44; both of which are conditions on which there is significantly more therapeutic evidence&#46; To a certain extent&#44; this is even the case nowadays&#46; To offer a clear example&#44; even today&#44; more than 70&#37; of patients with bronchiectasis are treated with long-acting bronchodilators&#44; although there is only one published clinical trial &#40;including fewer than 40 patients&#41; that supports their use&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Despite the above&#44; this scenario has gradually improved over the initial two decades of the 21st century&#46; At present there is a widely accepted definition of bronchiectasis &#40;not due to cystic fibrosis&#44; traction&#44; secondary to pulmonary interstitial processes&#44; nor emphysema&#41;&#44; as the abnormal bronchial dilatation resulting from a vicious pathogenic cycle consisting in chronic inflammation and airway repair&#46; This is usually accompanied by an infection by potentially pathogenic microorganisms &#40;PPMs&#41; and characteristic symptoms&#44; such as productive cough&#44; usually with a purulent component&#44; and exacerbations with an infectious profile&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">In short&#44; bronchiectasis is considered to be a form of pulmonary involvement of more than a hundred local and systemic diseases capable of causing chronic airway inflammation&#46; Even so&#44; its underlying cause remains unknown in over 25&#37;&#8211;35&#37; of cases&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">A critical point in the clinical evolution of bronchiectasis is the appearance of a bronchial infection caused by PPMs&#46; Infection by <span class="elsevierStyleItalic">Pseudomonas aeruginosa</span> &#40;especially its repeated isolation&#44; known as chronic bronchial infection&#41; clearly marks a turning point in the prognosis of patients with bronchiectasis&#46; It is not in vain that the relevant regulations coincide in pointing out that isolations of this microorganism in respiratory samples of patients with bronchiectasis &#40;generally in the sputum&#41;&#44; already during a first episode &#40;primary infection&#41;&#44; must be forcefully treated with antibiotics&#46; The main goal is to avoid its chronification&#44; especially when it also has a clinical impact on the patient &#40;usually an increase in the number and severity of exacerbations&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;8&#44;12&#44;13</span></a> In this sense&#44; excellent studies have been carried out in the context of bronchiectasis &#40;and cystic fibrosis&#41; research&#44; all demonstrating the beneficial effect of certain treatments for this condition &#40;and&#44; in general&#44; for chronic bronchial infection by any PPM causing an accelerated deterioration of the disease&#41;&#46; Clear examples of beneficial treatments include macrolides &#40;at immunomodulatory doses and&#44; therefore&#44; with an anti-inflammatory purpose&#41; and inhaled antibiotics&#44; of which there are already several available on the market and which achieve significant pulmonary deposition at the infection site&#44; with few associated systemic adverse effects&#44; thus allowing a prolonged but safe use&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">14</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">Another key aspect that has advanced in recent years is the determination of the relationship between bronchiectasis and other chronic inflammatory airway diseases&#44; such as COPD or asthma&#46; Several authors agree that between 30&#37; and 50&#37; of patients with severe COPD have bronchiectasis&#46; These areas of bronchiectasis usually have a cylindrical appearance&#44; are viewed in the lung bases&#44; and can only be detected by HRCT&#46; However&#44; their presence has been associated with more severe forms of COPD and a worse prognosis of this disease&#46; In fact&#44; some authors assert that bronchiectasis is part of the natural clinical history of COPD at advanced stages&#44; a presentation that has been termed the COPD-bronchiectasis phenotype&#46; Diagnosing this entity is crucial&#44; as its presence may imply a change in the patient&#8217;s therapeutic management&#46; Similarly&#44; up to 25&#37; of patients with severe asthma have bronchiectasis&#44; a presentation that is also associated with a greater number and severity of exacerbations&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a> This fact allows for appreciating the epidemiological importance of bronchiectasis&#44; which&#44; although clearly underdiagnosed&#44; is already the third most frequent chronic inflammatory airway disease&#44; after COPD and asthma&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">It is also worth noting the recent relationship observed between bronchiectasis and lung infection caused by the severe acute respiratory syndrome coronavirus 2 &#40;SARS-CoV-2&#41;&#46; Some studies agree that bronchiectasis can appear as a pulmonary sequela in up to 30&#37; of cases of pneumonia associated with the 2019 coronavirus disease &#40;COVID-19&#41;&#44; and in up to 80&#37; of those of severe pneumonia requiring admission to an intensive care unit &#40;ICU&#41;&#46; Although it is assumed that many of these forms of bronchiectasis are a result of traction secondary to pulmonary fibrotic scarring processes&#44; the truth is that their long-term clinical or prognostic impact on the patient is completely unknown and their study represents a great scientific challenge for the near future&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">An important fact that should be considered is that bronchiectasis is a heterogeneous and complex disease&#44;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> therefore&#44; it should not be considered as having exclusively pulmonary involvement&#44; but rather as what has come to be called <span class="elsevierStyleItalic">bronchiectatic syndrome</span>&#44; as the pulmonary inflammation inherent to the disease is often accompanied by systemic inflammation of varying nature&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;18</span></a> Hence&#44; management of patients with this condition should be multidisciplinary&#44; including pulmonologists&#44; internists&#44; radiologists&#44; nutritionists&#44; rehabilitators&#44; physiotherapists&#44; surgeons&#44; microbiologists&#44; or nurses&#44; among others&#44; but also primary care physicians during the clinical suspicion phase and for the management of patients with a chronic ailment&#46; This is because patients with bronchiectasis should not only be treated for their pulmonary infection and inflammation&#44; but also for their underlying etiological disease&#44; potential malnutrition&#44; exacerbations &#40;especially severe ones&#44; which are associated with a worse prognosis of the disease&#41;&#44;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">19</span></a> complications such as hemoptysis or respiratory failure&#44; and&#44; certainly&#44; palliative care in the terminally ill patient&#46; Therefore&#44; it seems advisable that this multidisciplinary team be integrated into specialized units&#44; at least for the management of more complex patients&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">However&#44; none of the above makes any sense if the health professional assessing these patients does not consider bronchiectasis as a differential diagnosis among all possible airway diseases&#46; A smoker with airflow obstruction should not always be diagnosed with COPD &#40;or with COPD alone&#41; nor should an atopic individual with wheezing always be diagnosed with asthma &#40;or with asthma alone&#41;&#46; Because this condition requires specific treatment&#44; an early diagnosis of bronchiectasis is crucial&#46; As mentioned earlier&#44; patients with bronchiectasis&#44; especially those with a bronchial infection or multiple exacerbations&#44; have been shown to benefit more from antiinflammatories&#44; macrolides &#40;at immunomodulatory doses&#41;&#44; or antibiotics &#40;especially inhaled&#41; than COPD and asthma patients&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">In short&#44; bronchiectasis was never an orphan or banal disease&#44; it simply remained occult until modern diagnostic imaging technology made it an identifiable condition&#46; Despite the wide therapeutic arsenal available today&#44; the number of bronchiectasis diagnoses continues to grow&#44; consequently entailing an increase in healthcare costs&#46; What seems even more paradoxical&#44; both the number of exacerbations and the mortality associated with this disease is increasing&#44; especially among older patients&#46; To conclude&#44; bronchiectasis should not be overlooked&#44; as not all airway conditions can be attributed to COPD or asthma&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors of this paper declare no conflicts of interest&#46;</p></span></span>"
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Article information
ISSN: 23870206
Original language: English
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

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Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos