Kembara Xtra - Medicine - Bronchiectasis Introduction Bronchiectasis is an uncurable dilatation of one of the airways that is characterized by persistent mucopurulent sputum production and recurring transmural bronchial infection or inflammation. It could be dispersed or focused. Epidemiology (Incidence and Prevalence) Predominant sex: female > male (1) Predominant age: most usually manifests in the sixth decade of life (1) Incidence Incidence has dropped in the United States for two reasons: Childhood respiratory infections can be effectively treated with antibiotics if they are caught early enough. Among children, incidence may be higher in indigenous or socioeconomically disadvantaged groups. Prevalence Prevalence in the United States is estimated to be 139/100,000; it is higher in women than in men (180 vs. 95/100,000); and it rises significantly with age (from 7/100,000 to 812/100,000 between the ages of 34 and 75 years). Pathophysiology and Etiology Chronic infections (PTB), autoimmune disorders, genetic reasons (cystic fibrosis (CF)), chronic obstructive pulmonary disease (COPD), connective tissue diseases, and allergic bronchopulmonary aspergillosis (ABPA) are among the conditions that are frequently idiopathic. Assuming a vicious circle Transmural infection, typically brought on by bacterial pathogens, results in airway inflammation and blockage. Inflammation and blockage are made worse by bacterial colonization, which is encouraged by damaged airways and defective cilia. Risk Factors Non-CF bronchiectasis has nontuberculous mycobacterial infection as both a cause and a consequence. Systemic illnesses, such as rheumatoid arthritis and inflammatory bowel disease, chronic rhinosinusitis, recurrent pneumonia, aspirated foreign bodies, immunodeficiency, congenital anomalies, severe respiratory infection in childhood (measles, adenovirus, influenza, pertussis, or bronchiolitis), chronic rhinosinusitis, and congenital abnormalities Prevention Routine vaccinations against pneumococcal pneumonia, measles, Haemophilus influenzae type B, influenza, and pertussis Smoking cessation and genetic counseling if a congenital issue is the cause. Accompanying Conditions Other congenital diseases include: Mucociliary clearance abnormalities, primary ciliary dyskinesia, Young syndrome (secondary ciliary dyskinesia), and Kartagener syndrome. - 1 antitrypsin deficiency - Marfan syndrome - Cartilage deficit (Williams-Campbell syndrome) - COPD - Pulmonary fibrosis, which results in traction bronchiectasis - Postinfectious conditions - Mycobacterial diseases (tuberculosis [TB] and Mycobacterium avium complex [MAC]), bacteria (H. influenzae and Pseudomonas aeruginosa), whooping cough, and Aspergillus species - Viral infections (HIV, adenovirus, measles, and influenza virus) Immune system disorders Hypogammaglobulinemia is the primary. - Supplemental: posttransplantation ABPA - Consequences of toxic inhalation or aspiration, such as luminal foreign bodies or chlorine. Chronic inflammatory rheumatic diseases - Sjögren syndrome - Rheumatoid arthritis Chronic granulomatous disease and inflammatory bowel disease Additional - Yellow nail syndrome Signs and Symptoms Chronic productive cough, wheezing, and dyspnea are typical symptoms, and recurring respiratory infections are frequently present. After a diagnosis, look into the cause. Presenting History Immunization history Any congenital, infectious, and/or exposure-related risk variables Symptoms are frequently persistent for a long time and include the following: - A persistent cough (90%) Sputum might be abundant and purulent (90%). Rhinosinusitis (60–70%) - Fatigue: a dominant symptom may exist (70%) Dyspnea (75%). - Pleuritic chest discomfort may be present (20–30%). Hemoptysis (20–30%) clinical assessment Fever may be a sign of aggravation. Asthma (20%) (60%) Bibasilar crackles (44%) Rhonchi Clubbing online (3%) Chest malformations could be present. Differential Diagnosis: Pulmonary TB, Asthma, Chronic Bronchitis, CF, COPD, and ABPA Diagnostic and Laboratory Tests Forced expiratory volume in the first second of expiration (FEV1): 80% expected and FEV1/FVC: 0.7 on spirometry, along with moderate airflow restriction and hyperresponsive airways - Additional testing - Electron microscopy-based ciliary biopsy H. influenzae, nontypeable form in sputum culture (42%) Aeruginosa (18%) - Streptococcus pneumoniae, Moraxella catarrhalis, MAC, and Aspergillus may also be detected in cultures. - Check selected people for TB and other diseases. – 30–40% of all isolates will not grow. Unique tests - A sweat test for CF; - A CBC with differential to ascertain if a main vs secondary immunological etiology may be present. - The PPD (purified protein derivative) TB test - Aspergillus skin testing; alternatively, Aspergillus antibodies (total IgE, IgG-specific antibodies, and IgE-specific antibodies) can be assessed. HIV, Serum Igs to check for humoral immunodeficiency, Protein electrophoresis to check for a lack of 1-antitrypsin, Barium swallow to check for abnormalities of deglutition, achalasia, and esophageal hypomotility, pH probe to check for reflux, and screening tests for rheumatologic illnesses chest computed tomography (CT) is the most effective diagnostic tool for primary biliary dyskinesia. - Bronchi are dilated and do not taper, resulting in the "tram track sign"; parallel opacities are visible on scan. - Chest radiograph is nonspecific; increased lung markings or may appear normal. - Balloon cysts and varicose constrictions may be present. – To rule out endobronchial blockage in cases with focal bronchiectasis. Consider CF and ABPA for upper lobe bronchiectasis alone. Other/Diagnostic Procedures Bronchoscopy can be used to remove sputum and collect cultures. Bronchoscopy for hemoptysis can help rule out lesions that block the airways and have focal bronchiectasis. Interpretation of Tests Among the results of bronchoscopy are the following: Thickened bronchial walls with necrosis of the bronchial mucosa, dilatation of the airways, and purulent discharges Management and Therapy Treat underlying medical issues. Identify a severe exacerbation using four of the nine criteria. - A modification in sputum production - Enhanced dyspnea - Enhanced cough - Fever - Enhanced wheeze - Malaise, weariness, and sluggishness - Decreased pulmonary function - Radiographic modifications - Variations in chest noises Non-CF bronchiectasis Identify the etiology of exacerbations and encourage excellent bronchopulmonary hygiene by clearing the airways every day. In the case of a focal disease that is unresponsive to medical treatment, think about removing the affected lung surgically. Patients with non-CF bronchiectasis may not respond to CF treatment regimens in the same manner that patients with CF do. Medical management: Lower morbidity by managing symptoms and stopping disease progression. The First Line of Medicine Antibiotics may be helpful in sudden exacerbations. - Sputum culture and sensitivity should guide therapy; a large variety of infections and resistant organisms confound antibiotic selection. – While awaiting culture results, empiric antibiotics could be started. Pseudomonas infection might be covered until the results come in. – For an acute exacerbation, patients may need double the usual dosage and prolonged treatment for 14 days (10 to 21 days). Ciprofloxacin for adults: 750 mg PO every 12 hours Trimethoprim sulfamethoxazole (SMX): 160 mg trimethoprim / 800 mg SMX PO q12h; pediatric: 2 months, 8 mg/kg trimethoprim and 40 mg/kg SMX PO/24 hours, administered in 2 divided doses q12h; Doxycycline and cefaclor administered PO are also effective. Macrolides: seem to be beneficial for immunomodulation Despite the fact that chronic therapy reduces sputum output, the amount of exacerbations, and hospitalizations, there is a chance that antibiotic resistance will develop. Adults who experience three or more exacerbations annually or who continue to experience substantial symptoms should receive long-term antibiotic therapy. P aeruginosa colonized: Colistin is the first line of treatment, followed by gentamicin, and long-term oral macrolides (azithromycin or erythromycin) should be considered in patients who cannot tolerate inhaled antibiotics or as a supplement to inhaled antibiotics in severe cases (ERS) (7). Long-term macrolide, doxycycline as a backup, and inhaled gentamicin if long-term oral therapy is unsuitable for non-P. aeruginosa colonized patients. - When administering macrolides to individuals who are at risk for QTc prolongation, proceed with caution. In the event of a serious infection, an IV should be given. Prior to starting chronic azithromycin therapy, all patients should be checked for non-TB mycobacterial infections. Limited research supports the use of bronchodilators in patients without breathlessness. – Before receiving physiotherapy, mucoactive medications, or inhaled antibiotics, use bronchodilators. Inhaled corticosteroids - Patients should not typically be prescribed inhaled corticosteroids unless they also have concurrent asthma or COPD (BTS/ERS). Mucoactive agents: Nebulized dornase and high-dose anti-inflammatory drugs like ibuprofen may be helpful in treating CF-related bronchiectasis, but they have no use in treating bronchiectasis that is not caused by the disease. In fact, people who use nebulized dornase may experience more frequent exacerbations and a loss in lung function. Mannitol may enhance quality of life and lessen sputum clogging (BTS). Nebulized saline or hypertonic saline (7% saline) use prior to airway clearance procedures can assist boost sputum output. Further Treatments Techniques for clearing sputum, such as postural drainage and percussion in chest physiotherapy, as well as pulmonary rehabilitation (which increases exercise tolerance) Surgery is an option if the bronchiectasis is localized, the symptoms are severe despite medical treatment, or the disease is life-threatening. In 80% of these situations, surgery successfully reduces symptoms. Motivation for Admission Massive hemoptysis that is life-threatening can be a symptom of bronchiectasis. In this case, bronchial artery embolization or surgical intervention is required to reduce bleeding in addition to airway preservation and resuscitation. Regular Treatment Children and adults with CF and non-CF associated bronchiectasis should be treated by comprehensive interdisciplinary chronic illness management programs, according to long-term outpatient therapy recommendations. Aim for normal growth and development in youngsters. Patients with CF should be sent to a CF center, and patients with main and secondary immunological deficits should get combined care with a clinical immunologist. The many methods for clearing the airways should be explained to the patient. Patients experiencing symptoms of dyspnea affecting daily activities should be provided with pulmonary rehabilitation. Use and alter antibiotics in the event of an acute exacerbation in accordance with sputum microbiology. Consider if long-term antibiotics are appropriate for patients who experience recurring exacerbations. Noninvasive ventilation can save hospitalizations and enhance quality of life in people with chronic respiratory failure. Consider performing a lung transplant examination on patients whose respiratory health is deteriorating and whose FEV1 is below 30%. However, distinct referral and listing criteria should be taken into consideration because non-CF bronchiectasis has a much lower mortality risk than CF-related bronchiectasis. Exercise on a regular basis is advised. patient observation Chest CTs to monitor disease development may be necessary for specific disorders, such as bronchiectasis with MAC infections. Serial spirometry should be performed at least once a year. routine sputum microbiologic analysis The mortality rate (the percentage of deaths directly attributable to bronchiectasis) is 10.6-29.7%. Poorer prognosis is linked to Pseudomonas infection, low body mass index, and advanced age. Hemoptysis, recurrent pulmonary infections, pulmonary hypertension, cor pulmonale, lung abscess, and complications
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