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Upper Airway ? 2: Bronchiectasis, Cystic Fibrosis and Sinusitis

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Upper Airway ? 2: Bronchiectasis, Cystic Fibrosis and Sinusitis Review series Upper airway ? 2: Bronchiectasis, cystic fibrosis and Thorax: first published as 10.1136/thx.2008.112870 on 1 December 2009. Downloaded from sinusitis M R Loebinger,1 D Bilton,2 R Wilson1 1 Host Defence Unit, Royal ABSTRACT inflammation damage the epithelium and stimu- Brompton Hospital, London, UK; The nose and paranasal sinuses are contiguous with the late excess mucus production, and mucosal swel- 2 Cystic Fibrosis Unit, Royal Brompton Hospital, London, UK lower respiratory tract. Patients with bronchiectasis and ling blocks the sinus ostia, further impairing mucus cystic fibrosis commonly have sinonasal disease, which is clearance. Bacterial infection and damage caused Correspondence to: thought to have the same aetiology and pathophysiology by the host inflammatory response interact, Dr R Wilson, Host Defence Unit, as the chronic lung disease. Despite this, the conditions perpetuating the disease process, and in chronic Royal Brompton Hospital, Sydney Street, London SW3 are rarely considered together and there is very little rhinosinusitis the disease can become independent 6NP, UK; [email protected] literature on the treatment of sinonasal disease in of continued bacterial infection. Similarly, bronchiectasis. In addition to being a common cause of impaired clearance from the lower airways leads Received 7 May 2009 comorbidity, there is evidence suggesting that sinonasal to chronic respiratory infection and inflammation Accepted 31 May 2009 disease may directly influence the bronchial condition. that has been termed a ‘‘vicious circle’’, leading to This article reviews sinonasal disease in bronchiectasis the progression of bronchiectasis.2 It is therefore and cystic fibrosis and addresses the possible interactions not surprising that bronchiectasis and sinusitis between the health and disease of the upper and lower often coexist. airways. Other important host defences in the upper respiratory tract include immunoglobulins, defen- sins and antibacterial components of the mucus The upper respiratory tract comprising the nose such as lysozyme. The defences can be impaired by and paranasal sinuses has the same mucosal lining primary or secondary abnormalities (table 1). The as the lower airways, and both compartments are upper respiratory tract is often exposed first to an involved together in health and disease. The infectious agent and to higher concentrations of relevance of this ‘‘single airway’’ concept has been inhaled matter and, for this reason, the initial demonstrated with asthma, allergic rhinitis and symptoms may come from this site and may be nasal polyposis, but it may be similarly useful in more severe. chronic infective respiratory conditions. Investigation of the upper airway may be useful SINONASAL DISEASE as a marker of disease in the less accessible lower The nasal cavity conditions the air we breathe, and http://thorax.bmj.com/ airway. In addition, treatment of one compart- it also acts as a first line of defence by impacting ment may have benefits throughout the whole unwanted particles on the mucosa and excluding respiratory tract. Improvements in sinus health them from the lower respiratory tract. The have been reflected in improved lower airway paranasal sinuses (frontal, maxillary, ethmoid and 1 disease in asthma. This has not been studied in sphenoid) are air-filled spaces within the skull. bronchiectasis or cystic fibrosis, but the implica- They reduce the weight of the skull and humidify tions are similar in that viral and bacterial and heat inhaled air. These cavities connect with on October 1, 2021 by guest. Protected copyright. infections can pass from one to the other. the nose via small orifices called ostia, which may Mucociliary clearance is an important first-line become blocked by swelling of inflamed mucosa. defence of both the upper and lower respiratory Rhinosinusitis is defined as inflammation of the tracts. The nose, sinuses, trachea and bronchi are nose and paranasal sinuses. Diagnostic criteria lined by ciliated and mucus-producing cells which include the presence of two or more symptoms form the mucociliary escalator and trap inhaled which should include one of nasal obstruction/ matter and propel it to the nasopharynx, where it congestion or anterior/posterior rhinorrhoea. can be expectorated or swallowed. Mucus trans- Other symptoms include anosmia and facial pain port depends on both the integrity and function of or pressure. The patient’s symptom score can the ciliated epithelium and the properties of the describe severity of disease on a visual analogue mucus. The cilia beat at a rate of between 12 and scale.3 Patients should also have evidence of 16 strokes per second in a consistent direction and rhinosinusitis on examination with polyps, muco- in a coordinated fashion. The periciliary fluid layer purulent discharge or oedema around the middle is of the required depth equalling the vertical meatus on nasendoscopy or mucosal changes height of a cilium, and the mucus must be of evident on the CT scan.4 suitable rheology for transport. Acute rhinosinusitis consists of symptoms last- Impaired sinonasal clearance leads to stasis of ing ,12 weeks, and can be subdivided into acute secretions, which are only removed from the nasal viral rhinosinusitis with a duration of ,10 days cavity by blowing or postnasal drip. This makes and acute non-viral rhinosinusitis with symptoms the host prone to bacterial infection which results lasting between 10 days and 12 weeks. in neutrophilic inflammation leading to chronic Chronic rhinosinusitis is defined by persistent rhinosinusitis. Bacterial products and neutrophilic symptoms lasting .12 weeks. It can be subdivided 1096 Thorax 2009;64:1096–1101. doi:10.1136/thx.2008.112870 Review series Table 1 Host defence abnormalities leading to chronic bacterial Table 2 Common causes of bronchiectasis and chronic rhinosinusitis Thorax: first published as 10.1136/thx.2008.112870 on 1 December 2009. Downloaded from infection of the upper and lower airways Prevalence of Host defence Primary Secondary chronic Disorder Examples rhinosinusitis References Cilia Kartagener’s syndrome Viral infection Primary ciliary dyskinesia Cigarette smoke Idiopathic 45–84% 11, 12 Mucus Young’s syndrome Viral infection Abnormal mucociliary PCD Up to 100% 13 clearance Cigarette smoke CF 14 Allergy Young’s syndrome 15 Periciliary fluid Cystic fibrosis Air conditioning Immunocompromise HIV Up to100% 16 Dehydration Common variable hypogammaglobulinaemia Antibacterial Common variable HIV immunodeficiency Postinfective Tuberculosis 50% 11 Unknown Idiopathic bronchiectasis Panbronchiolitis Non-tuberculous mycobacteria (eg, MAC) Yellow nail syndrome Whooping cough Ulcerative colitis Measles Excessive immune ABPA 40–90% 17 response GvHD Depends on by the presence or absence of nasal polyps. Nasal polyposis is a severity of chronic inflammatory disease of the nose and sinus mucosa. The bronchiectasis polyps occur when the oedematous lining of the nasal mucosa Inflammatory bowel disease becomes dependent, causing some obstruction of the nasal Mechanical Tumour Infrequent cavity. They can be found throughout the upper respiratory obstruction Foreign body tract but are most commonly located around the ostiomeatal Lymphadenopathy complex. Nasal polyposis is frequently associated with asthma, ABPA, allergic bronchopulmonary aspergillosis; CF, cystic fibrosis; GvHD, graft versus cystic fibrosis (CF), primary ciliary dyskinesia (PCD) and aspirin host disease; HIV, human immunodeficiency virus; MAC, mycobacterium avium sensitivity. Polyps are classified as: 0, no polyps; 1, mild (not complex; PCD, primary ciliary dyskinesia. reaching the upper edge of the inferior turbinate); 2, moderate (between the upper and lower edges of the inferior turbinate); characterised by excess mucus production and recurrent lower and 3, severe (reaching the lower edge of the inferior turbinate).5 respiratory tract infections. It is the end result of several The aetiology of chronic rhinosinusitis may be infective or non- different aetiologies (table 2). The most common causes of infective. The pathogenic role played by bacteria in chronic bronchiectasis are idiopathic and postinfective damage.10 11 rhinosinusitis is unknown. On the one hand they may be an Chronic rhinosinusitis occurs in 45–84% of patients with important stimulus of neutrophilic inflammation, whereas they idiopathic bronchiectasis11 12 and is less common in patients can also be seen as passengers taking advantage of the with postinfective bronchiectasis where an acute infection has 11 inflammatory milieu created by poor drainage from obstructed damaged the bronchial tree. It is almost universal, and often http://thorax.bmj.com/ ostia. The role of anaerobic bacteria in these circumstances is more severe, when the sinusitis and bronchiectasis share a also uncertain. It is also unknown whether the different common aetiology due to the host defence abnormalities listed sinonasal pathologies are independent diseases or part of a in table 1. These conditions include ciliary defects in PCD and spectrum with nasal polyps, the result of chronic rhinosinusitis mucous abnormalities in CF. which arises from an episode of acute rhinosinusitis. Complications of chronic sinusitis include mucocele formation Primary ciliary dyskinesis (PCD) which are cysts lined by respiratory epithelium. They occur in Each ciliated epithelial cell contains 50–200 motile cilia responsible the sinus cavities and can cause erosion of the surrounding bony for
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