Download MBBS Pulmonary Medicine Presentations 3 Bronchiectasis Lecture Notes

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&

LUNG ABSCESS

INTRODUCTION

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NOT COVERED

QUICK RUN

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CYSTIC FIBROSIS

THROUGH THE

CHILDREN 15

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LECTURE

YEARS

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DIAGNOSIS

ETIOLOGY

INVESTIGATIONS

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AND

MANAGEMENT
LEARNING OBJECTIVES

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? Definition
? Etiology
? Pathogenesis
? Clinical manifestations

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? Diagnosis
? Treatment

CASE 1

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? A 42-year-old man, gardener
? Long history of respiratory problems starting in early

childhood.

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? Previously diagnosed as asthma.
? Frequent absence from work due to "recurrent chest

infections".

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? Unaware of any neonatal issues but believes that he was born

at home without complications and is unsure of any previous

tests he has had as he is now estranged from his parents.

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? Has a cousin with a "lung disease".
? Married but has "no kids"

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INVESTIGATIONS

? Sputum culture: P. aeruginosa
? Sweat chloride = 73 meq/liter
? Cystic fibrosis genetics: genotype was F508del/R117H

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? CYSTIC FIBROSIS: Multisystem disorder caused by

mutations in the gene that encodes the CF

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transmembrane conductance regulator (CFTR) protein,

a chloride channel expressed in epithelial cells.

? More than 2000 CFTR mutations have been identified

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to date, but only the functional importance of a small

number is known to cause the disease
HRCT THORAX

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? An upper lobe predominant distribution of cylindrical, cystic and

varicose bronchiectasis associated with airway wall thickening, mucus

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plugging and parenchymal opacities on a HRCT scan should raise the

suspicion of CF disease.

? The presence of nasal polyposis and/or chronic rhinosinusitis,

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recurrent pancreatitis, malabsorption, diabetes, osteoporosis and

male infertility are other typical features of CF

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DIAGNOSIS

Guidelines published by the Cystic Fibrosis Foundation in the
USA allows diagnosis if:

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1. Clinical presentation of the disease and evidence of biochemical

and genetic markers of CFTR dysfunction.

2. Clinical features of the disease with concentration of chloride

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>60mmol?L-1 at the sweat test or a concentration in the

intermediate range (30?59mmol?L-1) but two disease-causing

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CFTR mutations.

3. CFTR genotype is undefined: CFTR physiologic tests, such as nasal

potential difference and intestinal current measurement, should

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be performed.
MANAGEMENT

1. CFTR modulator therapies

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2. Airway clearing techniques
3. Chest physical therapy
4. Humidification with sterile water or normal saline to facilitate

airway clearance

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5. Antibiotics
6. Mucus thinners
7. Lung transplantation

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CASE 2

? 45-year-old farmer with asthma since childhood.
? Complaints: Decline in his exercise tolerance and an

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increase in cough which has become productive of

purulent sputum with occasional thick/solid components.

? Respiratory exacerbations not responding well to

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standard steroid and antibiotic treatment.

? He was noted to have variable pulmonary infiltrates on

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chest radiographs during these episodes


INVSETIGATIONS

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? Marked peripheral blood eosinophilia
? Total IgE > 1000 IU/ ml
? Aspergillus specific IgE > O.35

ABPA: ABPA is an inflammatory disease caused by hypersensitivity to

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the ubiquitous fungus Aspergillus fumigatus
? ABPA occurs most commonly in patients with asthma and CF
? ABPA is the cause of 1?10% of cases of bronchiectasis
? Most ABPA cases occur in the third and fourth decade without a sex

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predilection.
DIAGNOSIS

? Long standing uncontrolled asthma/ Cystic fibrosis
? Brownish sputum

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? Peripheral eosinophilia > 500/ mm3
? Total IgE > 1000 IU/ ml
? Specific IgE for A. fumigatus > 0.35

HRCT thorax:

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? Central bronchiectasis
? High attenuation mucus
? Finger in glove/ TIB
? Tram track
? Mosaic attenuation

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MANAGEMENT

1. Corticosteroids
2. Antifungals

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3. Airway clearing techniques
4. Chest physical therapy
5. Mucus thinners

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CASE 3

? 77-year-old retired librarian.
? Cough for many years with new symptoms of fatigue, weight

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loss and fever.

? A chest CT scan was performed looking for a possible occult

malignancy and bronchiectasis was found.

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DIAGNOSIS

? HRCT thorax: cylindrical bronchiectasis and tree-in-bud

pattern in middle and lower lobes

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? Sputum for M. Tuberculosis: negative
? MGIT culture: MAC growth at 4 weeks
? Repeat MGIT: Positive for MAC
? Tests for immunodeficiency and ABPA: Negative

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MANAGEMENT

1. Management of NTM as per the organism and clinical

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picture

2. Airway clearing techniques
3. Chest physical therapy
4. Mucus thinners

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CASE 4

? A 66-year-old woman with established idiopathic

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bronchiectasis has had three to four exacerbations per year

for the past 3 years despite performing daily chest

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physiotherapy.

? Produces large volumes of sputum daily despite performing

the active cycle of breathing technique.

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? Testing for NTM, ABPA and other complications were

negative, but sputum shows persistent infection with P.

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aeruginosa.
? One of the most common presentations of bronchiectasis
? Exacerbations are one of the most important manifestations of

bronchiectasis and P. aeruginosa is the most frequent

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organism in severe bronchiectasis worldwide

? Cylindrical bronchiectasis is the most common morphological

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pattern identified on CT scans

MANAGEMENT

1. Review current airway clearance regime.

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2. Repeat sputum microbiology and repeat testing for NTM, ABPA

and ensuring the all possible treatable causes and comorbidities

have been identified.

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3. First-line recommendation for P. aeruginosa with frequent

exacerbations is an inhaled antibiotic.

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ETIOLOGY

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INVESTIGATIONS FOR CAUSE

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? COMORBIDITIES AND RELEVANT PAST HISTORY

? FULL BLOOD COUNT/ SERUM TOTAL IGE/ SKIN PRICK TEST TO A. FUMIGATUS

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? SERUM Ig G/ IgA/ IgM

IN ALL

? BASELINE SPECIFIC ANTIBODY LEVELS AGAINST CAPSULAR POLYSACCHRIDES OF STREPTOCOCCUS

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PNEUMONIAE

? SPUTUM CULTURE : ROUTINE AND MYCOBACTERIAL

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CLINICALLY

STABLE

? HIV

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? TEST FOR CYSTIC FIBROSIS/ PCD/ GERD

? RA, ANTI CCP , ANCA, ANA

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CLINICALLY ? ALPHA 1 AT

SUSPECT ? BRONCHIAL ASPIRATION OR WASH

Hill A, Welham S, Sullivan A, Loebinger M. Updated BTS Adult Bronchiectasis Guideline 2018: a multidisciplinary

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approach to comprehensive care. Thorax. 2018;74(1):1-3.

STEPWISE MANAGEMENT

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AIRWAY CLEARANCE


Physiotherapy management-stepwise airway clearance.

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ACBT


POSTURAL DRAINAGE

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Airway clearance - exacerbations.


ANTIBIOTIC TREAMENT

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FOR EXACERBATION


WHAT IS THE ROLE OF

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SURGERY IN MANAGING

BRONCHIECTASIS?

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RECOMMENDATIONS
? Consider lung resection in patients with localized disease whose

symptoms are not controlled by medical treatment optimized by a

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bronchiectasis specialist. (D)

? Offer multidisciplinary assessment, including a bronchiectasis

physician, a thoracic surgeon and an experienced anesthetist, of

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suitability for surgery and pre-operative assessment of

cardiopulmonary reserve post resection. (D)
LUNG TRANSPLANTATION

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FOR BRONCHIECTASIS

Recommendations
? Consider transplant referral in bronchiectasis patients aged 65

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years or less if the FEV1 is <30% with significant clinical instability

or if there is a rapid progressive respiratory deterioration despite

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optimal medical management. (D)

? Consider earlier transplant referral in bronchiectasis patients with

poor lung function and the following additional factors: massive

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haemoptysis, severe secondary pulmonary hypertension, ICU

admissions or respiratory failure (particularly if requiring NIV).(D)

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LUNG ABSCESS
DEFINITION

Localized area of lung suppuration, leading to

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necrosis of the lung parenchyma with or without

cavity formation.

Type of liquefactive necrosis of the lung tissue

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and formation of cavities (more than 2 cm)

containing necrotic debris or fluid caused by

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microbial infection.

ACUTE

DURATION

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CHRONIC

PRIMARY

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LUNG ABSCESS

AETIOLOGY

SECONDARY

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BRONCHOGENIC

WAY OF

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SPREADING

HEMATOGENIC
CLASSIFICATION (CONTD.)

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? ACCORDING TO THE DURATION:

? Acute (less than 6 weeks);

? Chronic (more than 6 weeks)

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? BY ETIOLOGY:

? Primary (aspiration of oropharyngeal secretions, necrotizing

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pneumonia, immunodeficiency);

? Secondary (bronchial obstructions, haematogenic dissemination,

direct spreading from mediastinal infection, from sub phrenic space,

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coexisting lung diseases)

? WAY OF SPREADING:

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? Bronchogenic (aspiration of oropharyngeal secretions, bronchial

obstruction by tumour, foreign body, enlarged lymph nodes,

congenital malformation);

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? Haematogenic (abdominal sepsis, infective endocarditis, septic

thromboembolisms)

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DIFFERENTIATING ACUTE

FROM CHRONIC

ACUTE

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CHRONIC

CIRCUMSCRIBED

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IRREGULAR

NOT WELL DEFINED

WELL DEFINED

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FILLED WITH NECROTIC

DEBRIS

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FILLED WITH THICK DEBRIS


DIFFERENTIAL DIAGNOSIS

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? Excavating bronchial carcinoma (squamo-cellular or microcellular)
? Excavating tuberculosis
? Localized pleural empyema
? Infected emphysematous bullae
? Cavitary pneumoconiosis

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? Hiatus hernia
? Pulmonary hematoma
? Hydatid cyst of lung
? Cavitary infarcts of lung
? Wegener's granulomatosis

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DIAGNOSIS

? Diagnostic bronchoscopy is a part of diagnostic protocol

for taking the material for microbiological examination

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and to confirm intrabronchial cause of abscess-tumor or

foreign body.

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? Sputum examination is useful for identification of

microbiological agents or confirmation of bronchial

carcinoma

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MANAGEMENT

STANDARD CONSERVATIVE THERAPY: MEDICAL MANAGEMENT
? It is recommended to treat lung abscess with broad spectrum

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antibiotics, due to poly microbial flora, such as Clindamycin (600

mg IV on 8 h) and then 300 mg PO on 8 h or combination

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ampicilin/sulbactam (1.5-3 gr IV on 6 h).

? Alternative therapy is piperacilin/tazobactam 3.375 gr IV on 6 h or

Meropenem 1 gr IV on 8 h.

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? For MRSA it is recommended to use linezolid 600 mg IV on 12 h or

vancomycin 15 mg/kg BM on 12 h.
MANAGEMENT

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SURGICAL
? Endoscopic drainage of lung abscesses is described as an alternative to chest

tube drainage and is performed during the bronchoscopy with usage of laser.

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? Per cutaneous trans thoracic tube drainage
? Surgical resection of lung abscess is the therapy of choice for about 10% of

patients.

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? Lobectomy is the resection of choice for large or central position of abscess.

Atypical resection or segmentectomy are satisfactory procedures, if it is

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possible to remove complete abscess and if necessary surrounding lung tissue

with necrotizing pneumonia

THANK YOU

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BRONCHIECTASIS

DEFINITION

? Bronchiectasis (broncos, airways; ectasia, dilatation) is a

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morphologic term used to describe abnormal,

irreversibly dilated and thick walled bronchi.

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? This is an anatomic definition that evolved from

Laennec's original description in 1819 of ectatic bronchi

in pathologic specimens.

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La?nnec RTH. On mediate auscultation, or a treatise on the diagnosis of diseases of the lungs and heart. Paris: J.-A.

Brosson et J.-S. Chaud?; 1819.
PREVALENCE

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US1

UK2

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INDIA

? Prevalence increased

? Prevalence in women

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? There is no good data on

every year from 2000 to

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566/lakh ; men= 486/lakh

bronchiectasis in India

2007 by an annual

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? Women and age more

? EMBARC INDIA REGISTRY

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percentage change of

than 60 years associated

(European Multi Centre

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8.74%.

with higher rate of

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Bronchiectasis Audit and

? Increased with age

hospitalization

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Research Collaboration)

(peak= 80-84 years)

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? Higher in women

1. McShane P, Naureckas E, Tino G, Strek M. Non?Cystic Fibrosis Bronchiectasis. American Journal of Respiratory and Critical Care

Medicine. 2013;188(6):647-656.

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2. Hil A, Welham S, Sullivan A, Loebinger M. Updated BTS Adult Bronchiectasis Guideline 2018: a multidisciplinary approach to

comprehensive care. Thorax. 2018;74(1):1-3.

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BURDEN

LONGER HOSPITAL STAY

FREQUENT OPD VISITS

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INCREASED EXPENDITURE ON

MEDICINES

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RISK FACTORS FOR MORTALITY

LOW FEV1

MALE

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MORTALITY RATE= 10-16%

INCREASED DYSPNOEA GRADE

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ADVANCED AGE

COPD

P. aeruginosa SPUTUM POSITIVITY

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? Goeminne PC, Scheers H, Decraene A, Seys S, Dupont LJ. Risk factors for morbidity and death in non?cystic fibrosis bronchiectasis: a

retrospective cross-sectional analysis of CT diagnosed bronchiectatic patients. Respir Res 2012;13:21.

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? Weycker D, Edelsberg J, Oster G, Tino G. Prevalence and economic burden of bronchiectasis. Clin Pulm Med 2005;12:205?209.
PATHOGENESIS

COLES'S VISCIOUS CYCLE MODEL

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PULMONARY

INFECTION/

TISSUE INJURY

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ROBUST

BACTERIAL

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INFLAMMATORY

COLONIZATION

RESPONSE

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AIRWAYS

ABNORMAL

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DESTRUCTION

MUCUS

AND

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CLEARANCE

DISTORTION

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Cole PJ. Inflammation: a two-edged sword--the model of

bronchiectasis. Eur J Respir Dis Suppl 1986;147:6?15.

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PATHOLOGY

Hematoxylin and eosin stain of the bronchial

wall

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in a patient with bronchiectasis (left) versus a

normal subject (right).

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A: Pseudostratified columnar, ciliated

epithelium

B: thickened epithelium with intraepithelial

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lymphocytes

C: submucosa with dense infiltrate of

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lymphocytes and plasma cells

D: blood vessel with reactive endothelial

cells.

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BRONCHIECTATIC

NORMAL

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TYPES

REIDS

CLASSIFICATION

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CYLINDRICAL

VARICOSE

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SACULAR/CYSTIC

Reid LM. Reduction in bronchial subdivision in bronchiectasis. Thorax

1950;5:233?247.

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TYPES OF BRONCHIECTASIS

ETIOLOGY

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IN WHOM TO

SUSPECT ?

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PERSISTENT PURULENT OR MUCO

PURULENT SPUTUM

+

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IN HEALTHY?1

COPD FREQUENT

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HIV/ SOLID ORGAN OR

EXACERBATORS WITH

BONE MARROW

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SPUTUM CULTURE

TRANSPLANT/

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IBD/ GERD/ CTD/

RA WITH RECURRENT

SEVERE/ POORLY

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POSITIVE FOR P.

IMMUNOSUPRRESIVE

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RHINOSINUSITIS

CHEST INFECTIONS

CONTROLLED ASTHMA

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AERUGINOSA WHEN

THERAPY FOR

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STABLE

LYMPHOMA OR

VASCULITIS

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Hil A, Welham S, Sullivan A, Loebinger M. Updated BTS Adult Bronchiectasis Guideline

2018: a multidisciplinary approach to comprehensive care. Thorax. 2018;74(1):1-3.

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ALGORITHM FOR

EVALUATION OF

BRONCHIECTASIS

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Cantin L, Bankier A, Eisenberg R. Bronchiectasis. American Journal of Roentgenology.

2009;193(3):W158-W171.

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INVESTIGATION: RADIOLOGY

? BASELINE CHEST RADIOGRAPH
? THIN SECTION CT [HRCT THORAX]

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CT FEATURES OF BRONCHIECTASIS

vBRONCHIAL DILATATION SUGGESTED BY

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? BRONCHOARTERIAL RATIO >1 (INTERNAL AIRWAY LUMEN VS ADJACENT PULMONARY ARTERY)

? LACK OF TAPERING

? AIRWAY VISIBILITY WITHIN 1CM OF COSTAL PLEURAL SURFACE OR TOUCHING MEDIASTINAL

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PLEURA.

vINDIRECT SIGNS

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? BRONCHIAL WALL THICKENING

? MUCUS IMPACTION

? MOSAIC PERFUSION / AIR TRAPPING ON EXPIRATORY CT

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CHEST RADIOGRAPH

CYLINDRICAL

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BRONCHIECTASIS

WITHIN I CM OF

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PLEURA

TRAM TRACK SIGN

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SIGNET RING SIGN

VARICOSE

BRONCHIECTASIS

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VARICOSE

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CYSTIC BRONCHIECTASIS

MUCUS IMPACTION

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CYSTIC

CLUSTERS

MOSAIC PERFUSION

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BLACK

WHITE
INVESTIGATIONS FOR CAUSE

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? COMORBIDITIES AND RELEVANT PAST HISTORY

? FULL BLOOD COUNT/ SERUM TOTAL IGE/ SKIN PRICK TEST TO A. FUMIGATUS

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? SERUM Ig G/ IgA/ IgM

IN ALL

? BASELINE SPECIFIC ANTIBODY LEVELS AGAINST CAPSULAR POLYSACCHRIDES OF STREPTOCOCCUS

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PNEUMONIAE

? SPUTUM CULTURE : ROUTINE AND MYCOBACTERIAL

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CLINICALLY

STABLE

? HIV

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? TEST FOR CYSTIC FIBROSIS/ PCD/ GERD

? RA, ANTI CCP , ANCA, ANA

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CLINICALLY ? ALPHA 1 AT

SUSPECT ? BRONCHIAL ASPIRATION OR WASH

Hill A, Welham S, Sullivan A, Loebinger M. Updated BTS Adult Bronchiectasis Guideline 2018: a multidisciplinary

--- Content provided by FirstRanker.com ---

approach to comprehensive care. Thorax. 2018;74(1):1-3.

STEPWISE MANAGEMENT

--- Content provided by‌ FirstRanker.com ---

AIRWAY CLEARANCE


Physiotherapy management-stepwise airway clearance.

--- Content provided by​ FirstRanker.com ---

ACBT


POSTURAL DRAINAGE

--- Content provided by‍ FirstRanker.com ---

Airway clearance - exacerbations.


ANTIBIOTIC TREAMENT

--- Content provided by⁠ FirstRanker.com ---

FOR EXACERBATION


WHAT IS THE ROLE OF

--- Content provided by​ FirstRanker.com ---

SURGERY IN MANAGING

BRONCHIECTASIS?

--- Content provided by‍ FirstRanker.com ---

RECOMMENDATIONS
? Consider lung resection in patients with localized disease whose

symptoms are not controlled by medical treatment optimized by a

--- Content provided by‍ FirstRanker.com ---

bronchiectasis specialist. (D)

? Offer multidisciplinary assessment, including a bronchiectasis

physician, a thoracic surgeon and an experienced anesthetist, of

--- Content provided by⁠ FirstRanker.com ---

suitability for surgery and pre-operative assessment of

cardiopulmonary reserve post resection. (D)
LUNG TRANSPLANTATION

--- Content provided by FirstRanker.com ---

FOR BRONCHIECTASIS

Recommendations
? Consider transplant referral in bronchiectasis patients aged 65

--- Content provided by​ FirstRanker.com ---

years or less if the FEV1 is <30% with significant clinical instability

or if there is a rapid progressive respiratory deterioration despite

--- Content provided by​ FirstRanker.com ---

optimal medical management. (D)

? Consider earlier transplant referral in bronchiectasis patients with

poor lung function and the following additional factors: massive

--- Content provided by‌ FirstRanker.com ---

haemoptysis, severe secondary pulmonary hypertension, ICU

admissions or respiratory failure (particularly if requiring NIV).(D)

--- Content provided by⁠ FirstRanker.com ---

LUNG ABSCESS
DEFINITION

Localized area of lung suppuration, leading to

--- Content provided by⁠ FirstRanker.com ---

necrosis of the lung parenchyma with or without

cavity formation.

Type of liquefactive necrosis of the lung tissue

--- Content provided by​ FirstRanker.com ---

and formation of cavities (more than 2 cm)

containing necrotic debris or fluid caused by

--- Content provided by​ FirstRanker.com ---

microbial infection.

ACUTE

DURATION

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CHRONIC

PRIMARY

--- Content provided by⁠ FirstRanker.com ---

LUNG ABSCESS

AETIOLOGY

SECONDARY

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BRONCHOGENIC

WAY OF

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SPREADING

HEMATOGENIC
CLASSIFICATION (CONTD.)

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? ACCORDING TO THE DURATION:

? Acute (less than 6 weeks);

? Chronic (more than 6 weeks)

--- Content provided by⁠ FirstRanker.com ---

? BY ETIOLOGY:

? Primary (aspiration of oropharyngeal secretions, necrotizing

--- Content provided by‌ FirstRanker.com ---

pneumonia, immunodeficiency);

? Secondary (bronchial obstructions, haematogenic dissemination,

direct spreading from mediastinal infection, from sub phrenic space,

--- Content provided by⁠ FirstRanker.com ---

coexisting lung diseases)

? WAY OF SPREADING:

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? Bronchogenic (aspiration of oropharyngeal secretions, bronchial

obstruction by tumour, foreign body, enlarged lymph nodes,

congenital malformation);

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? Haematogenic (abdominal sepsis, infective endocarditis, septic

thromboembolisms)

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DIFFERENTIATING ACUTE

FROM CHRONIC

ACUTE

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CHRONIC

CIRCUMSCRIBED

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IRREGULAR

NOT WELL DEFINED

WELL DEFINED

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FILLED WITH NECROTIC

DEBRIS

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FILLED WITH THICK DEBRIS


DIFFERENTIAL DIAGNOSIS

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? Excavating bronchial carcinoma (squamo-cellular or microcellular)
? Excavating tuberculosis
? Localized pleural empyema
? Infected emphysematous bullae
? Cavitary pneumoconiosis

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? Hiatus hernia
? Pulmonary hematoma
? Hydatid cyst of lung
? Cavitary infarcts of lung
? Wegener's granulomatosis

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DIAGNOSIS

? Diagnostic bronchoscopy is a part of diagnostic protocol

for taking the material for microbiological examination

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and to confirm intrabronchial cause of abscess-tumor or

foreign body.

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? Sputum examination is useful for identification of

microbiological agents or confirmation of bronchial

carcinoma

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MANAGEMENT

STANDARD CONSERVATIVE THERAPY: MEDICAL MANAGEMENT
? It is recommended to treat lung abscess with broad spectrum

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antibiotics, due to poly microbial flora, such as Clindamycin (600

mg IV on 8 h) and then 300 mg PO on 8 h or combination

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ampicilin/sulbactam (1.5-3 gr IV on 6 h).

? Alternative therapy is piperacilin/tazobactam 3.375 gr IV on 6 h or

Meropenem 1 gr IV on 8 h.

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? For MRSA it is recommended to use linezolid 600 mg IV on 12 h or

vancomycin 15 mg/kg BM on 12 h.
MANAGEMENT

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SURGICAL
? Endoscopic drainage of lung abscesses is described as an alternative to chest

tube drainage and is performed during the bronchoscopy with usage of laser.

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? Per cutaneous trans thoracic tube drainage
? Surgical resection of lung abscess is the therapy of choice for about 10% of

patients.

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? Lobectomy is the resection of choice for large or central position of abscess.

Atypical resection or segmentectomy are satisfactory procedures, if it is

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possible to remove complete abscess and if necessary surrounding lung tissue

with necrotizing pneumonia

THANK YOU

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CASE 1

? A 42-year-old man, gardener

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? Long history of respiratory problems starting in early

childhood.

? Previously diagnosed as asthma.

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? Frequent absence from work due to "recurrent chest

infections".

? Unaware of any neonatal issues but believes that he was born

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at home without complications and is unsure of any previous

tests he has had as he is now estranged from his parents.

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? Has a cousin with a "lung disease".
? Married but has "no kids"
INVESTIGATIONS

? Sputum culture: P. aeruginosa

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? Sweat chloride = 73 meq/liter
? Cystic fibrosis genetics: genotype was F508del/R117H

? CYSTIC FIBROSIS: Multisystem disorder caused by

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mutations in the gene that encodes the CF

transmembrane conductance regulator (CFTR) protein,

a chloride channel expressed in epithelial cells.

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? More than 2000 CFTR mutations have been identified

to date, but only the functional importance of a small

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number is known to cause the disease

HRCT THORAX

? An upper lobe predominant distribution of cylindrical, cystic and

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varicose bronchiectasis associated with airway wall thickening, mucus

plugging and parenchymal opacities on a HRCT scan should raise the

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suspicion of CF disease.

? The presence of nasal polyposis and/or chronic rhinosinusitis,

recurrent pancreatitis, malabsorption, diabetes, osteoporosis and

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male infertility are other typical features of CF
DIAGNOSIS

Guidelines published by the Cystic Fibrosis Foundation in the

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USA allows diagnosis if:

1. Clinical presentation of the disease and evidence of biochemical

and genetic markers of CFTR dysfunction.

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2. Clinical features of the disease with concentration of chloride

>60mmol?L-1 at the sweat test or a concentration in the

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intermediate range (30?59mmol?L-1) but two disease-causing

CFTR mutations.

3. CFTR genotype is undefined: CFTR physiologic tests, such as nasal

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potential difference and intestinal current measurement, should

be performed.

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MANAGEMENT

1. CFTR modulator therapies
2. Airway clearing techniques
3. Chest physical therapy

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4. Humidification with sterile water or normal saline to facilitate

airway clearance

5. Antibiotics

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6. Mucus thinners
7. Lung transplantation


CASE 2

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? 45-year-old farmer with asthma since childhood.
? Complaints: Decline in his exercise tolerance and an

increase in cough which has become productive of

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purulent sputum with occasional thick/solid components.

? Respiratory exacerbations not responding well to

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standard steroid and antibiotic treatment.

? He was noted to have variable pulmonary infiltrates on

chest radiographs during these episodes

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INVSETIGATIONS

? Marked peripheral blood eosinophilia
? Total IgE > 1000 IU/ ml
? Aspergillus specific IgE > O.35

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ABPA: ABPA is an inflammatory disease caused by hypersensitivity to
the ubiquitous fungus Aspergillus fumigatus
? ABPA occurs most commonly in patients with asthma and CF
? ABPA is the cause of 1?10% of cases of bronchiectasis

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? Most ABPA cases occur in the third and fourth decade without a sex

predilection.

DIAGNOSIS

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? Long standing uncontrolled asthma/ Cystic fibrosis
? Brownish sputum
? Peripheral eosinophilia > 500/ mm3
? Total IgE > 1000 IU/ ml

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? Specific IgE for A. fumigatus > 0.35

HRCT thorax:
? Central bronchiectasis
? High attenuation mucus

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? Finger in glove/ TIB
? Tram track
? Mosaic attenuation
MANAGEMENT

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1. Corticosteroids
2. Antifungals
3. Airway clearing techniques
4. Chest physical therapy
5. Mucus thinners

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CASE 3

? 77-year-old retired librarian.
? Cough for many years with new symptoms of fatigue, weight

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loss and fever.

? A chest CT scan was performed looking for a possible occult

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malignancy and bronchiectasis was found.


DIAGNOSIS

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? HRCT thorax: cylindrical bronchiectasis and tree-in-bud

pattern in middle and lower lobes

? Sputum for M. Tuberculosis: negative

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? MGIT culture: MAC growth at 4 weeks
? Repeat MGIT: Positive for MAC
? Tests for immunodeficiency and ABPA: Negative
MANAGEMENT

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1. Management of NTM as per the organism and clinical

picture

2. Airway clearing techniques

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3. Chest physical therapy
4. Mucus thinners

CASE 4

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? A 66-year-old woman with established idiopathic

bronchiectasis has had three to four exacerbations per year

for the past 3 years despite performing daily chest

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physiotherapy.

? Produces large volumes of sputum daily despite performing

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the active cycle of breathing technique.

? Testing for NTM, ABPA and other complications were

negative, but sputum shows persistent infection with P.

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aeruginosa.


? One of the most common presentations of bronchiectasis

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? Exacerbations are one of the most important manifestations of

bronchiectasis and P. aeruginosa is the most frequent

organism in severe bronchiectasis worldwide

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? Cylindrical bronchiectasis is the most common morphological

pattern identified on CT scans
MANAGEMENT

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1. Review current airway clearance regime.
2. Repeat sputum microbiology and repeat testing for NTM, ABPA

and ensuring the all possible treatable causes and comorbidities

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have been identified.

3. First-line recommendation for P. aeruginosa with frequent

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exacerbations is an inhaled antibiotic.



THANK YOU

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