Other autoimmune diseases such as ulcerative colitis and Crohns disease also have an association with bronchiectasis. Additionally, graft-versus-host disease in patients who have undergone stem cell transplantation can lead to bronchiectasis as well. Lung injury
Bronchiectasis could be caused by: inhalation of ammonia and other toxic gases, chronic pulmonary aspiration of stomach acid from esophageal reflux, or a hiatal hernia. Congenital
Bronchiectasis may result from congenital disorders that affect cilia motility or ion transport. A common genetic cause is cystic fibrosis, which affects chloride ion transport. Another genetic cause is primary ciliary dyskinesia, a rare disorder that leads to immotility of cilia and can lead to situs inversus. When situs inversus is accompanied by chronic sinusitis and bronchiectasis, this is known as Kartageners syndrome. Other rare genetic causes include Youngs syndrome and Williams-Campbell syndrome. Tracheobronchomegaly, or Mournier-Kuhn syndrome is a rare condition characterized by significant tracheobronchial dilation and recurrent lower respiratory tract infections. Individuals with alpha 1-antitrypsin deficiency have been found to be particularly susceptible to bronchiectasis, due to the loss of inhibition to enzyme elastase which cleaves elastin. This decreases the ability of the alveoli to return to normal shape during expiration. Cigarette smoking
A causal role for tobacco smoke in bronchiectasis has not been demonstrated. Nonetheless, tobacco smoking can worsen pulmonary function and accelerate the progression of disease that is already present.
The impact of adverse effects of macrolides such as gastrointestinal symptoms, hepatotoxicity, and increased antimicrobial resistance needs ongoing review and study.Inhaled corticosteroid therapy can reduce sputum production and decrease airway constriction over a period of time, helping prevent progression of bronchiectasis. Long term use of high-dose inhaled corticosteroids can lead to adverse consequences such as cataracts and osteoporosis. It is not recommended for routine use in children. One commonly used therapy is beclometasone dipropionate. Antibiotics
Antibiotics are used in bronchiectasis to eradicate P. aeruginosa or MRSA, to suppress the burden of chronic bacterial colonization, and to treat exacerbations. The use of daily oral non-macrolide antibiotic treatment has been studied in small case series, but not in randomized trials. The role of inhaled antibiotics in non-CF bronchiectasis has recently evolved with two society guidelines and a systematic review suggesting a therapeutic trial of inhaled antibiotics in patients with three or more exacerbations per year and P. aeruginosa in their sputum. Options for inhaled antibiotics include aerosolized tobramycin, inhaled ciprofloxacin, aerosolized aztreonam, and aerosolized colistin. Bronchodilators
Some clinical trials have shown a benefit with inhaled bronchodilators in certain people with bronchiectasis. In people with demonstrated bronchodilator reversibility on spirometry, the use of inhaled bronchodilators resulted in improved dyspnea, cough, and quality of life without any increase in adverse events. However, overall there is a lack of data to recommend use of bronchodilators in all patients with bronchiectasis.