What is Bronchiectasis? Broncos, airways: ectasia, dilation Bronchiectasis is a condition anatomically defined by chronic, irreversible dilation and distortion of the bronchi and bronchioles caused by inflammatory destruction of the muscular and elastic components of the bronchial walls. It is not a primary disease but secondary to persistent infections or obstruction caused by several conditions.
Etiology Bronchiectasis is not a primary disease but rather secondary to persisting infection or obstruction caused by a variety of conditions. It is basically due to necrotizing inflammation with damage to the airway wall. For example, when patient have chronic inflammation and infection within the airway which results in damage to the wall which then dilates the airway. The conditions that most commonly predispose to bronchiectasis include: Cystic Fibrosis: An inherited condition, cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.
Kartagener Syndrome: It is a defect in dynin arm. Inherited as an autosomal recessive disease, immotile cilia syndrome can lead to bronchiectasis, as a result of recurrent pulmonary infections due to retained secretions. It can lead to sinusitis, Bronchiectasis, and situs invertus. Bronchial Obstruction: The most common causes are tumors foreign bodies and occasionally impaction of mucus. Infections: Virulent organisms such as staphylococcus aureus or Klebshiella may predispose affected patients to development of bronchiectasis. Allergic Broncho pulmonary Aspergillosis: Allergic Broncho pulmonary aspergillosis is associated with airway damage and bronchiectasis. Several factors may lead to this, including direct invasion of the airways by the fungus, immune reaction to aspergillus.
Immune Dysfunction: Immunodeficiency syndromes such as immunoglobulin deficiency, complement deficiency and chronic granulomatous disease, are associated with bronchiectasis. Deficiency of IgG, IgM and IgA, put the patient at increased risk of recurrent pulmonary infections, that eventually end in bronchiectasis.
Pathogenesis There is either chronic persistent infection occurs or obstruction can occur. Either one can come first. Due to the obstruction, the normal clearance mechanisms are hampered, and soon secondary infection will follow. Over time, chronic infection can causes damage to the bronchial walls leading to weakening and dilation. A persistent necrotizing inflammation in the bronchi or bronchioles can cause obstructive secretions and inflammation through out the wall.
TYPES Pathologically, bronchiectasis can be divided into four types. The first type, cylindrical bronchiectasis, is characterized by uniform dilatation of bronchi, that extends into the lung periphery. The second type is called varicose bronchiectasis and is characterized by irregular and beaded outline of bronchi, with alternating areas of constriction and dilatation. The third type is called cystic or saccular bronchiectasis and is the most severe form of the disease. The bronchi dilate, forming large cysts, which are usually filled with air and fluid. The fourth type of bronchiectasis is called follicular and is characterized by extensive lymphoid nodules within the bronchial walls. It usually occurs following childhood infections
Clinical Presentation Patient presents with cough because of the mucous that is trapped in these larger airways. Dyspnea Production of large quantities of purulent and often foul smelling sputum. People with bronchiectasis may have bad breath indicative of active infection. Bronchiectasis may also present with Hemoptysis, coughing up blood, in the absence of sputum Other symptoms include, fatigue, weight loss, wheezing, Complications: Hypoxemia because there is trapping of CO2, Cor pulmonale, and secondary amyloidosis.
Diagnosis And Treatment Chest Radiograph: Tram lines( dilated and thick airways), increase pulmonary markings. Signet rings Ct Scan: Train Track sign ( The bronchial wall is thicken and visible) Ring sign ( Dilated bronchi appeared as ring structures Sputum culture Complete blood count Bronchoscopy (To assess the cause of hemoptysis, and to evaluate the proximal airway for lesion)
Treatment: Treatment is aimed at controlling infections and bronchial secretions, relieving airway obstruction, and preventing complications. Antibiotics, bronchodilators, and expectorants are often prescribed for infections. Surgery