Bronchiectasis

No bronchiectasis today, just revising random conditions that I need to learn for this year, and looking back fondly over my time on Resp…

So, I’m sat in the TB clinic with my firm partner, Adam, in between patients with the Reg who’s running the clinic. And I can’t remember how the conversation got there, but Adam says, “I cough up blood sometimes“, like it’s totally normal or something. And the Reg, who is running a clinic for TB-a red flag symptom of which is haemoptysis- says, “really…?” And I’m like (in my head)- “it’s cause he smokes, totally cause he smokes, ask him if he smokes Reg.” And the Reg, with a smile that says “I know something but I’m not telling you” then asks him if he often has a cough- yes– and if he got frequent chest infections as a child-yes- and the whole time, I’m like- “just ask him if he smokes Reg, just ask him!” (in my head, of course). Then the Reg says, “I would say based on that history that you’ve probably got bronchiectasis. I actually have bronchiectasis myself. You should go and see your GP about it.” And so Adam is all weirded out because he thought it was nothing and I’m kind of disappointed because I was looking forward to Adam getting told of for smoking by a respiratory registrar, and feeling kind of left out because I don’t have bronchiectasis.


Bronchiectasis

Definition: chronic infection of the bronchi and bronchioles leading to permanent dilatation of these airways.

Image result for types of bronchiectasisAetiology/ risk factors:

-Abnormal and permanent dilatation of the central and medium-sized airways leads to impaired clearance of bronchial secretions with secondary bacterial infection and bronchial inflammation. It may be localised to a lobe or generalised throughout the bronchial tree.

Main organisms: H.influenzae, Strep.pneumoniae, Staph.aureus, Pseudomonas aeruginosa

Causes:

Congenital: cystic fibrosis, Young’s syndrome, primary ciliary dyskinesia (PCD), Kartagener’s syndrome (= immotile cilia (PCD), situs invertus, chronic sinusitis, infertility, bronchiectasis).

Post-infection: bronchial damage following measles, pertussis (whooping cough), bronchiolitis, pneumonia, TB, HIV (immune deficiency).

Other: bronchial obstruction (foreign body, tumour), allergic bronchopulmonary aspergillosis (ABPA), hypogammaglobulinaemia (immune deficiency- reduced antibodies), rheumatoid arthritis, ulcerative colitis.

Many cases are idiopathic (~50%)

Epidemiology: ~ 1 in 1000 per year

most often arises initially in childhood

-incidence decreased with use of antibiotics

Symptoms:

-persistent, chronic productive cough

-copious thick, purulent (green/yellow) sputum = recurrent chest infections

-intermittent haemoptysis (like Adam!) (may be massive and life-threatening)

-breathlessness and wheeze (maybe)

-other symptoms: chest pain, malaise, fever, weight loss

-symptoms usually begin after an acute respiratory illness

Signs:

-finger clubbing

coarse inspiratory crepitations- over affected area, usually lung bases, shift with coughing

-wheeze (if asthma, COPD, ABPA)

Investigations:

Sputum culture– essential during an infective exacerbation. The common organisms are H.influenzae, Strep.pneumoniae, Staph.aureus, Pseudomonas aeruginosa.

Chest X-ray: may be normal or show dilated bronchi with thickened bronchial walls, and sometimes multiple cysts containing fluid (air-fluid levels may be seen in cystic bronchiectasis). May also show fibrosis, atelectasis or pneumonic consolidation.

-tramline- airways perpendicular to x-ray beam, parallel lines radiating from hilum to diaphragm, seen in cylindrical bronchiectasis- and ring shadows-airways seen on end.

High resolution CT: Gold Standard for diagnosis. Can assess extent and distribution of disease. Shows thickened, dilated airways, and bronchial wall cysts that may not be shown on standard CXR.

Spirometry: often show’s obstructive pattern (FEV1:FVC < 75%)

Bronchoscopy: to locate site of haemoptysis, exclude obstruction, obtain samples for culture

-Bronchography- rarely used, to determine extent of disease before surgery. Radio-opaque contrast injected through the cricoid ligament or via a bronchoscope.

Image result for bronchography bronchiectasis

Other tests: serum immunoglobulins (hypogammaglobulinaemia?), cystic fibrosis sweat test (conc. of chloride in sweat elevated),  Aspergillus precipitins or skin-prick test.

Management:

-Advise smoking cessation.  Annual influenza and pneumococcal vaccinations.

Postural drainage 2x/day. Chest physiotherapy may aid sputum expectoration and mucus drainage.

Antibiotics– for exacerbations, according to bacterial sensitivities (Ciprofloxacin for Pseudomonas) . If ≥3 exacerbations per year, consider long-term antibiotics.

Bronchodilators (e.g. nebulised Salbutamol)- may be useful in patients with asthma, COPD, ABPA, CF. May provide symptomatic relief.

Corticosteroids (e.g. prednisolone)- for ABPA. Inhaled or oral steroids can decrease rate of progression of bronchiectasis.

Surgery– e.g. local resection, may be indicated in localised disease or to control severe haemoptysis. Severe disease sometimes requires lung or heart-lung transplantation.

-May consider bronchial artery embolisation- for life threatening haemoptysis

Complications:

-Pneumonia

-Pleural effusion

-Empyema

-Pneumothorax

-Haemoptysis- may be massive

-Respiratory failure

-Cor pulmonale

-Metastatic cerebral abscess

-Amyloidosis

Prognosis: irreversible condition, most patients continue to have the symptoms after 10 years

References: Cheese & Onion, Kumar and Clarke’s, Rapid Medicine, Radiopaedia.org
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