Download Bronchiectasis

Bronchiectasis
Yoon Jung Oh,M.D.
Departments of Pulmonary and
Critical Care Medicine
Ajou University School of Medicine
Definition
Abnormal and permanent dilatation of medium
sized( ≥ 2mm) bronchus by destruction of muscular,
cartilage and elastic tissue component
Three patterns of bronchiectasis (by Reid in 1950)
⑴ Cylindrical bronchiectasis
⑵ Cystic bronchiectasis
⑶ Varicose bronchiectasis
Involved site
⑴ Lt.lower lobe post.basal segment (common)
⑵ Rt.middle lobe
⑶ bilateral (30%)
Case
M/30
상기 30세 남환은 수일 전 감기를 앓은 후 내원 당일 고열과 화
농성 객담을 동반한 기침을 주소로 내원하였다.
환자는 부비동염으로 이비인후과에서 치료를 자주 받았다하며,
1년전에도 폐렴으로 입원한적이 있었다 한다. 평소 간헐적인
객혈이 있었으나 결핵의 기왕력은 없었다.
P/E moist crackles on left lower lung field
Bronchiectasis
Cystic Bronchiectasis
Cystic Bronchiectasis
Bronchogram
HRCT
Bronchiectasis
Cylindrical Bronchiectasis
Varicose Bronchiectasis
Pathogenesis and Predisposing Factors
• Bronchopulmonary infections
• Hereditary abnormalities
• Immunodeficiency states
• Bronchial obstruction
• Congenital anatomic defects
• Miscellaneous disorders
연령에 따른 원인
Childhood
Post pneumonia (Measle, Pertussis, Tbc)
1. Mechanical obstruction(foreign body)
2. Cystic fibrosis
3. -globulin deficiency(cong./aquired)
4. Immotile cilia syndrome
Adulthood
1.
2.
3.
4.
5.
Post pneumonia(viral, aspiration)
Pulmonary tuberculosis
Asthma, ABPA(allergic bronchopulmonary aspergillosis)
Toxic fume or congenital
Neoplasm, Chagas’ disease
Bronchopulmonary infections
• Childhood diseases
pertussis; measles
• Other bacterial infections
S.aureus, Klebsiella, M.tuberculosis, H.influenaze
• Other viral infections
adenovirus, influenza, H.simplex, HIV
• Miscellaneous infections
Mycotic infections
Mycolplasmal infections
Nontuberculous mycobacteria
Hereditary Abnormalities
• Ciliary defects of respiratory mucosa
Kartagener’s syndrome
: bronchiectasis, situs inversus, sinusitis
Immotile cilia syndrome
: male infertility, defect in dynein arm or radical spoke
• 1-Antitrypsin deficiency
: panacinar emphysema, bronchiectasis
• Cystic fibrosis
Kartagener’s Syndrome
• Triad
bronchiectasis, sinusitis, situs inversus
• Incidence : 1/68,000
• Inheritance : Autosomal recessive trait
• Pathogenesis
* absent or defective dysnein arms
* functional abnormality
(e.g., perhaps dysnein ATPase)
Kartagener’s syndrome
Immotile Cilia Sydrome
Diagnostic Criteria
1. Recurrent & Chronic URI and LRI
: rhinitis, sinusitis, otitis, bronchitis, bronchiectasis
2. Absence or near absence of tracheobronchial or nasal
mucociliary transport
3. Total or near-total absence of dynein arms of the cilia in
nasal or bronchial mucosa
4. Sterility in males
Normal Cilia
MT : microtubules
C : central sheath
R : radial spokes
D : dysnein arms
N : nexin
Schematic cross section views of cilia
A : Normal cilium
Nine outer pairs of MT
around a central pair
B – D : congenital ciliary defects
B : lacking dysnein arms
C : missing radial spokes
D : abnormal transposition
Tuberculosis and Bronchiectasis
1. Marked degree of caseation necrosis of bronchial walls,
particularly when upper lobes are invaded
2. Scarring of larger bronchi can produce bronchial stenosis
3. Extraluminal obstruction of larger bronchi by tuberculous
hilar lymphadenopathy
4. Penetration by a calcified tuberculous node into the airway
and broncholith formation
5. Healed tuberculous cavities
Clinical Features of Bronchiectasis
Symtoms
Signs
sputum
crackles
chronic cough
finger clubbing
hemoptysis
cyanosis
dyspnea
cor pulmonale
fever
chest pain
Diagnosis
• History / Physical examination
• Plain chest X-ray : normal finding in 20%
• HRCT(high resolution CT)
• Bronchogram
Indications
① Preoperative evaluation of unilateral or segmental
disease previously identified on CT
② Postoperativ evaluation of surgical airway complications
such as dehiscence or fistula formation
Differential Diagnosis
• Chronic bronchitis
• Endobronchial tuberculosis
• Endobronchial adenoma
• Right middle lobe syndrome
• Cystic fibrosis
• Allergic bronchopulmonary aspergillosis
• Pulmonary sequestration
• Congenital abnormalities of the trachiobronchial tree
Aim of Treatment
1. Elimination of an identifiable underlying problem
2. Improved clearance of tracheobronchial secretions
3. Control of infection
4. Reversal of airflow obstruction
Treatment : Medical
1. Treatment of predisposing factors
hypogammaglobulinemia  administration
immunoglobulin
tuberculosis  anti-tbc medication
ABPA  steroid
2. Bronchial drainage : most important
PDPV(postural drainage, percussion, vibration)
PEEP
deep brathing, continued coughing
3. Antibiotics
Treatment : Surgical
Indication
Contraindication
Localized bronchiectasis
Diffuse fibrosis or COPD
Massive/frequent hemoptysis
Bilateral bronchiectasis
Intractable infection
Bronchiectasis complicating asthma
Long standing collapse
Low FEV1/FVC
Adequate residual lung function
Persisting predisposing factors
(e.g. immunodeficiency)
Complications
• Recurrent pneumonia, sinusitis
• Lung abscess
• Pleuritis, empyema
• Emphysema
• Brain abscess(rare, but 10% of mortality)
• Amyloidosis
• Cor pulmonale(common in Korea)
• Massive hemoptysis(rare)
Cystic Fibrosis
• Incidence : Caucasian 1/1,500 – 2,000
Asian
1/90,000
• Inheritance : Autosomal recessive trait
• Pathogenesis
Single gene disorder
defective c-AMP mediated regulation of chloride channels
-> failure to secete chloride toward the lumen
-> unusually viscid mucous secretions
-> chronic pulmonary disease and pancreatic insufficiency
Cystic fibrosis
Diagnosis of cystic fibrosis
1. Sweat chloride test
> 60 mEq : confirmative
50 – 60 mEq : highly probable
< 50 mEq : normal
2. Chronic pulmonary disease (99%)
3. Pancreatic insufficiency(80-90%)
4. CXR
increased interstitial marking (98%)
cystic bronchiectasis (64%)
hyperinflation(58%)
Treatment of cystic fibrosis
Infection control
pseudomonas(80%) S.aureus, H.influenza
Bronchial drainage
postural drainage, percussion and vibration
forced exhalation technique
positive expiratory pressure