BronchiectasisJ47

Unilateral or bilateral, localized or diffused over the lung parenchyma (usually basal in the lower lobes), irreversible, sack-shaped or cylindrical dilatation of the bronchi with destruction of the bronchial wall.

Acquired:

Postinfectious:

• Bacterial infections (Staphylococcus, Pseudomonas aeruginosa, Haemophilus influenza, Mycobacteria, Bordatella pertussis.
• Viral infections: HIV, measles, influenza, adenovirus
• Fungal infections: histoplasm, coccidiomycosis, allergic bronchopulmonary aspergillosis(ABPA)

COPD

Pulmonary fibrosis, post-tuberculous fibrosis, radiation fibrosis

Compression of the bronchial tubes by space-occupying processes (lymphadenopathy, benign or malignant tumours)

Acquired immunological defects: tumour-associated, chemotherapy, long-term immunosuppressive therapy

Amyloidosis, celiac disease

Bronchiectasis In the context of syndromal diseases:

Yellow nail syndrome

Young syndrome (azoospermia, sinubronchial syndrome, bronchiectasis)

Congenital:

Primary Ciliary Dyskinesia (PCD)

Anatomical defects: Scoliosis, Marfan syndrome, tracheobronchomegaly

Cystic fibrosis

Cartagenic Syndrome

Alpha1-antitrypsin deficiency

Defective ENaC protein

Congenital immunological defects: Primary: CVID (variable immunodeficiency syndrome, agammaglobulinemia, hyper-IgE syndrome)

Incidence: 10/100,000 inhabitants/year; prevalence: 67/100,000 inhabitants

Necrotizing inflammatory processes in chronic bronchial infections. The bronchial secretion constantly accumulates in the sacculations and dilatations of the bronchi. The consequences of this are congestion-related, recurrent infections with microbial colonisation of the secretion and recurrent chronic inflammatory processes.

Congenital or acquired at a later age

Chronic cough with (mouthful) purulent sputum. The sputum increases in the course of the disease. Frequent detection of bacteria. 75% of those affected complain of shortness of breath (dyspnoea), 50% of those affected complain of haemoptysis (coughing up blood). Auscultatory: localized coarse-bubbled noises over the lungs.

Recurrent infections (symptoms: increase in sputum, increased dyspnea. fever, wheezing, fatigue, deterioration in lung function). Visible signs of chronic hypoxia: cyanosis, flail fingers and watch glass nails. Chronic lung failure can develop over many years.

Note: Three layer sputum: mucus-pus blood

Clinical findings. Diagnostic imaging. X-ray thorax in 2 planes (possibly ring shadow detectable); high-resolution computer tomography of the thorax (HR-CT) with direct detection of bronchiectases.

Blood gas analysis

Quantitative determination of immunoglobulins (immunoglobulin deficiency syndromes)

Bacteriological examination of sputum with antibiogram.

Development of chronic obstructive bronchitis (COPD)

Chronic right heart failure

Lung abscess

Amyloidosis

In the foreground of the treatment is the consistent treatment of the infections by targeted antibiotic administration. Necessity of a higher dosage and a longer therapy time (compared to uncomplicated infections of the respiratory tract). Possibly permanent or regularly recurring antibiotic doses are necessary (if necessary also by inhalation: topramycin, colistin have proven to be effective).

Mucolytics and mucus liquefiers are controversial but useful as adjuvants (most preparations lack proof of efficacy for long-term therapy).

For COPD - bronchospasmolytic therapy (see below bronchitis chronic obstructive pulmonary disease)

Yearly active immunization against influenza and at larger intervals against pneumococcus recommended.

In case of very localized bronchiectasis (only in one pulmonary lobe) or complications (threatening hemoptysis, fungal infection), surgical removal of the bronchiectasis may be considered.

In case of uncontrollable haemoptysis, surgical closure of the supplying blood vessel.

With progressing process . Development of respiratory insufficiency

Ultima ratio: Lung transplantation

The disease usually progresses chronically; it is treatable but not curable. Exception: Individual cases where surgical removal of the bronchiectasis is possible).

In the course of the disease, recurrent respiratory tract infections occur, which spread to the surrounding lung tissue over the years and lead to its destruction. Regular antibiotic therapy can significantly reduce the course of the disease.

Physiotherapy to mobilize secretions from the dilated bronchi (secretion drainage - bronchial toilet).

Various techniques to be performed independently, such as breathing exercises, positional drainage/tapping massages/vibration massages (best performed in knee-elbow position).

Autogenous drainage" (huffing): Secretions are coughed up by means of special breathing exercises.

Further: inhalation measures to mobilize the secretion.

Abundant hydration: helps to keep the secretion fluid to facilitate coughing.

Recommended for patients: http://lungenstiftung.de/

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2. Brower KS et al (2014) The etiologies of non-CF bronchiectasis in childhood: a systematic review of 989 subjects. BMC Pediatr 14:4.
3. Coutinho D et al. (2016) Surgical treatment of bronchiectasis: A review of 20 years of experience. Rev Port Pneumol 22: 82-85.
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5. Shi ZL et al.(2014) Effectiveness and safety of macrolides in bronchiectasis patients: a meta-analysis and systematic review. Pulse Pharmacol Ther 28:171-178.
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7. Wu Q et al (2014) Long-term macrolides for non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis. Respirology 19:321-329.