Montantes

Sir, this patient has got bronchiectasis affecting both lower lobes as evidenced by late, coarse inspiratory crepitations heard best posteriorly in the lower one third bilaterally. Patient has a productive cough with large volume of purulent sputum with hemoptysis associated with clubbing.

Chest excursion was reduced bilaterally with a normal percussion note and vocal resonance. Trachea is central and the apex beat is not displaced.

There are no signs to suggest presence of COPD.

(There is concomitant COPD with a reduced chest excursion bilaterally, hyperinflation of the chest associated with hyperresonance on percussion with loss of liver and cardiac dullness. There is presence of ronchi and a prolonged expiratory phase. Vocal resonance is normal. Trachea is central and apex beat is not displaced.)

There is complication of pulmonary hypertension with a loud and palpable component of the second heart sound associated with a left parasternal heave. There is also cor pulmonale with a raised JVP of 3 cm with prominent a wave associated with bilateral pedal oedema. Clinically there are no signs of polycythemia such as plethoric facies or conjunctival suffusion.

He is not in respiratory distress (with a RR of 14 bpm without use of accessory muscles of respiration). There are no signs of respiratory failure (he does not require any supplemental oxygen and there is no central cyanosis; there is also no flapping tremor of the hands and no bounding pulse). There is also no nicotine staining of the fingers, patient is not cachexic looking and no enlarged Cx LNs.

With regards to aetiology, there is no dextrocardia or a nasal voice to suggest possible Kartagener‟s syndrome. In addition, there is no symmetrical deforming polyarthropathy to suggest RA or any cutaneous signs of SLE. There is no kyphoscoliosis.

With regards to treatment, patient has a steroid metered-dose inhaler, salbutamol and ipratropium metered-dose inhalers by the bed side.

I would like to complete the examination by looking at the temperature chart for fever as well as an abdominal examination to look for splenomegaly from amyloidosis which can result from bronchiectasis. A neurological examination is useful to screen for deficit as patients are prone to brain abscesses.

In summary, this patient has bronchiectasis affecting both lower lobes with complications of pulmonary hypertension and cor pulmonale. There is no concomitant COPD and no polycythemia. He is clinically not in respiratory failure. The possible causes for this patient‟s bronchiectasis are post infective causes such as post viral, bacterial, TB or ABPA, connective tissue disease such as RA or SLE, congenital conditions such as cystic fibrosis, Kartagener‟s syndrome or hypogammaglobulinemia.

Questions

What are your differential diagnoses for a patient that is clubbed and has crepitations?

o Bronchiectasis o Pulmonary fibrosis o Mitotic lung lesion o Abscess

What is bronchiectasis?

o Definition: permanent dilatation of the bronchi

o Pathology: Retained secretions and chronic inflammation

o Clinical course: Chronic, progressive with recurrent infective exacerbations

o Clinical: Symptoms - productive purulent cough, dyspnea and hemoptysis and Signs: coarse late inspiratory crepitations with a 3 layered purulent sputum

What are the causes of bronchiectasis?

o Focal

o Luminal blockage – FB, broncholith

o Arising from the wall – mitotic lesion of the lung

o Extrinsic – enlarged LNs esp middle lobe from TB/fungi; displacement of airways post lobar resection o Diffuse

o Post infectious conditions

Bacteria – Pseudomonas, Hemophilus, Pertussis TB

Aspergillus (for upper lobe or proximal bronchiectasis) as in allergic bronchopulmonary aspergillosis from type III immune complex reactions.

Virus – adenovirus, measles, influenza o Congenital conditions

Cystic fibrosis

Alpha 1 Antitrypsin deficiency

Kartagener‟s syndrome of immotile ciliary syndrome Hypogammaglobulinemia

o NB: Immunodeficiency form secondary causes such as cancer, chemotherapy or immune modulation post transplant

o Rheumatic conditions

RA (1-3% of patients) SLE

Sjogren‟s o Others

Yellow nail syndrome (yellow nails, bronchiectasis, pl effusion and lymphedema) Young‟s syndrome(secondary ciliary dyskinesia from mercury intoxication) Inflammatory bowel disease (UC or Crohn)

Congenital kyphoscoliosis Idiopathic (50%)

What is bronchiectasis sicca?

o “dry” bronchiectasis

o Presents with recurrent hemoptysis and dry cough o Affects the upper lobes therefore good drainage

o Usually from past history of granulomatous infection eg TB What is Kartagener‟s syndrome?

o It is a type of immotile ciliary syndrome o Comprising of

o dextrocardia, situs inversus

o bronchiectasis, sinusitis, frontal sinus dysplasia, otitis media o infertility

o Resulting in poor ciliary function with retained secretions and recurrent infections and thus bronchiectasis What is cystic fibrosis?

o Most commonly due to mutations to CFTR (CF transmembrane conductance regulator) with F508 o Recurrent respiratory infections with pancreatic exocrine deficiency and short stature

o Upper lobe involvement o Staph aureus, Ps aeuroginosa

o Elevated sweat Na and Cl concentrations What are the differences in bronchiectasis vs COPD?

o They may both occur concomitantly

COPD Bronchiectasis

Cause Cigarette Infection, genetic

Infection Secondary Primary

Organism S. pneumoniae, Haem Haem, Pseudomonas

Symptoms Dyspnea, chronic cough Dyspnea, hemoptysis, productive

Sputum Mucoid clear 3 layered, purulent

CXR Hyperlucency, hyperinflated Airway thickening, dilated

28 What are the complications of bronchiectasis?

o Pneumonia, collapse, pleural effusion, lung abscess, pneumothorax, hemoptysis o Brain abscess

o Sinusitis o Amyloidosis How would you investigate?

The diagnostic investigation of choice is a HRCT but simple Ix such as CXR and LFT are also useful:

o CXR – Diagnosis, extent and complications o 90% abnormal

o Diagnosis specific

dilated and thickened airways Ring shadows (seen on end) Tram lines

Non-specific

Linear or plate-like atelectasis Scattered irregular opacities Focal pneumonitis

o Extent and distribution o Complications

Pneumonia, abscesses, pleural effusion o Lung function test

o Obstructive pattern with FEV1/FVC <70%

o Severity of obstruction based on FEV1 o Reversibility with beta agonist

40% of patients have >15% improvement o High-resolution computer tomography scan of the thorax

o Non-contrast study with 1 mm cuts every 1 cm with acquisition time of one second during full inspiration (requires patient cooperation); 90% sensitivity

o Diagnostic

Dilatation of airway lumen >1.5X cf to a nearby vessel Signet ring sign (dilated bronchus with its pulmonary artery)

Lack of tapering of an airway toward the periphery with presence of bronchi within 1 cm from the pleura Reid‟s classifications

Cylindrical or tubular Varicose

Saccular or cystic

Useful also in elucidation cause of focal bronchiectasis o Assess distribution

Usually lower lobes

If upper lobes – suspect Cystic fibrosis or ABPA If proximal bronchiectis, ABPA

If ML or lingula – M. avium complex o Complications

How would you manage?

o Non-Pharmacological

o Education and counselling

o Stop smoking, vaccinations (yearly influenza and 3-yearly pneumococcal) o Chest percussion and postural drainage (no evidence actually)

o Rx underlying cause o Pharmacological

o Rx acute exacerbations

o O‟Donnell‟s 4/9 symptoms of exacerbations Increased dyspnea

Increase cough

Increase sputum production Increased wheezing Fever

Lethargy, malaise Changes in chest sounds Reduced pulmonary function

Radiographic changes consistent with a new pulmonary process o Antibiotics targeting

Haem, Ps and Strep and Moraxella Fluoroquinolones

Others

MAC – Rifampiciin, ethambutol and Azithro till c/s negative for 1 year

ABPA – augmentation of corticosteroids and use of itraconazole 200mg bd for 4 weeks then 200mg om for 4 more weeks

o Bronchodilator therapy such as beta agonists and anticholinergics with inhaled corticosteroids o Improve lung function (FEV1) and reduce sputum volume

o No effect on mortality

o Aerosolised recombinant human DNAse for cystic fibrosis (not for other causes of bronchiectasis) o Surgery

o Focal

o Removal of obstructing tumour or FB o Diffuse

o Segments that are most damaged and contributing to recurrent acute exacerbations o Segments involved with uncontrolled haemorrhage

o Removal of segments suspected of harbouring drug resistant organism such as MDR MTB or MAC o Lung transplant

How do you manage complication of hemoptysis?

o Quantify

o If >600mls /day = massive o Lie on the affected side

o Protect airway

o Bronchoscope or CT to determine site of bleed o Interventional radiology or surgical removal

30 15. Interstitial Lung Disease

Presentation

Sir, this patient has interstitial lung disease affecting both lower lobes (upper lobes) as evidenced by fine velcro-like late inspiratory crepitations heard best posteriorly(anteriorly) in the lower one third bilaterally. This is associated with clubbing(50%) and a non-productive cough.

Chest excursion was reduced bilaterally with a normal percussion note and vocal resonance. Trachea is central and apex beat is not displaced.

There are no signs of pulmonary hypertension or cor pulmonale. There are also no features of polycythemia.

Patient respiratory rate is 14 breaths per minute and there are no signs of respiratory distress. There are also no signs of respiratory failure. There is also no nicotine staining of the fingers and I note that the patient is cachexic looking with wasting of the temporalis muscles.

In terms of aetiology, there is no symmetrical deforming polyarthropathy of the hands to suggest RA, or cutaneous signs to suggest presence of SLE, dermatomyositis or scleroderma as these conditions may be complicated by pulmonary fibrosis.

With regards to treatment, patient is not Cushingoid and does not have papery thin skin or steroid purpura to suggest chronic steroid usage. On inspection there are no surgical scars to suggest open lung biopsy.

I would like to complete the examination by asking for a detailed drug history as well as an occupational history.

In summary, this patient has got pulmonary fibrosis affecting bilateral lower lobes. There are no complications of pulmonary hypertension, cor pulmonale and polycythemia. He is clinically not in respiratory failure and has no features of chronic steroid usage. The differential diagnoses include collagen vascular disease, drugs, occupational causes and idiopathic pulmonary fibrosis.

Questions

What are the differential diagnoses for clubbing and crepitations?

Pulmonary fibrosis Bronchiectasis Lung abscess Mitotic lung conditions

What are the characteristic auscultatory findings?

Late, fine inspiratory crepitations Velcro-like

Disappears or quietens with the patient leaning forwards What are the causes of fibrosis?

Upper Lobes

S – Silicosis, sarcoidosis C- coal worker pnemoconiosis H- histiocytosis

A- Ankylosing spondylitis, ABPA R – radiation

I – Idiopathic pulmonary fibrosis O- others ie drugs

Cytotoxics – MTX, Aza, bleomycin, bulsulphan, cyclo, chlorambucil CNS - Amitryptyline, phenytoin and carbamazepine

CVS - Amiodarone, hydralazine, procainamide Antibiotics - Nitrofurantoin, isoniazid

Antirheumatics – Gold, sulphasalazine Both

N – Neurofibromatosis, Tuberous sclerosis

E – Extrinsic allergic alveolitis (acute symptoms within 6 hrs of inhaled allergens eg farmer‟s lungs) P – pulmonary haemorrhage syndromes

A – alveolar proteinosis Primary

Secondary – Inhaled organic dusts(Silica, Al), chronic infection, malignancy Lymphangiomyomatosis

How would you classify interstitial lung disease? (ATS/ERS 2001) Diffuse parenchymal lung disease(DPLD) of known cause

Collagen Vascular disease

RA, SLE, Dermatomyositis, Systemic sclerosis Occupational/Environmental

Asbestosis, silicosis, extrinsic allergic alveolitis Drug related

Cytotoxic, CNS, CVS, Antibiotics and antirheumatic Idiopathic

IPF

Other idiopathic interstitial pneumonias DIP

AIP LIP NSIP

Cryptogenic organising pneumonia Respiratory bronchiolitis

Granulomatous Sarcoidosis

Others - LAMs, histiocytosis

How would you diagnose idiopathic pulmonary fibrosis?

Clinical-radiological-pathological diagnosis Clinical

o Exclusion of other causes of ILD

o >50 yrs, insidious onset of dyspnea, > 3months, non-productive cough o Typical physical findings

Radiological (see below) Pathological (see below) How would you investigate?

The diagnostic Ix of choice is a HRCT of the thorax but simple IX such as CXR and LFT are useful:

CXR

Diagnostic

bilateral basal reticulonodular shadows, peripheries, which advances upwards honeycombing in advanced cases (gps of closely set ring shadows)

loss of lung volume Extent and distribution Complications Lung function

Restrictive pattern (reduced TLC or VC with increased FEV1/FVC ratio) Severity of restriction based on TLC

Reduced transfer factor (impaired gas exchange) HRCT scan

Dx – patchy reticular abnormalities, focal ground glass, architectural distortion, volume loss, subpleural cyst, honeycombing (no consolidation or nodules)

Extent and severity – basal, peripheral, subpleural Complications

NB: Similar to that of collagen vascular disease and asbestosis Others

Bronchoscopy – lavage

Predominantly lymphocyte responds to steroids and better Px= not UIP

Predominantly neutrophils and eosinophils means poor Px= UIP (if >20% of eosinophils to consider eosinophilic lung disease)

Lung biopsy

IPF – Usual interstitial pneumonia Bloods

ABGs

To rule out causes How would you manage?

Education and counselling Stop smoking

Regular follow up and vaccinations Treat underlying cause

Pharmacological Trial of steroids

If responding continue steroids

If not responding, cyclophosphamide or azathioprine Antifibrotic agents

Eg penicillamine which has not been proven to be useful Surgical: Lung transplant (single lung transplantation)

Manage complications

Cor pulmonale - diuresis for heart failure Polycythemia - venesection if Hct >55%

Respiratory failure – Oxygen therapy Monitor for lung cancer

32 What are the good prognosticating factors?

Young age Female Short duration

Ground glass appearance on the CXR Minimal fibrosis on lung biopsy

What is the clinical course of patients with IPF?

Gradual onset Progressive

Median survival from time of dx about 3 years What are the causes of death?

Cor pulmonale Respiratory failure Pneumonia Lung carcinoma

What is Hamman-Rich syndrome?

Rapidly progressive and fatal variant of interstitial lung disease

16. COPD

In document Proyecto estructural de un edificio industrial en el polígono industrial Cavila, Caravaca de la Cruz (página 160-178)