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BRONKIEKTASIS
dr. Aisyah Radiyah, Sp.P
 DEFINISI
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 Bronchiectasis (broncos, airways; ectasia, dilatation) is a morphologic
term used to describe abnormal, irreversibly dilated and thick- walled
bronchi
 Laennec’s original  (1819)
 Bronchiectasis represents the end stage of a variety of pathologic
processes that cause destruction of the bronchial wall and its
surrounding supporting tissues
 Adalah dilatasi abnormal bronkus yang kronik dan menetap disertai
destruksi dinding bronkus akibat kelainan kongenital ataupun yang
didapat seperti infeksi kronik saluran napas

(1) Oxford handbook of respiratory medicine 2021

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4 EPIDEMIOLOGI

 Seitz dkk: prevalensi meningkat 8.74% di Amerika Serikat selama

2004-2007, puncaknya usia 80-84 th, lebih banyak dijumpai pada
wanita, dan ras Asia
 UK, 2013: prevalesi 500/100.000
 Prevalensi meningkata seiring peningkatan usia. Prevalensi usia >60
th 300-500/100.00 dibanding 40-50/100.000 di usia 40-50 th
 Indonesia?

(1) Oxford handbook of respiratory medicine 2021

(2) Weycker D, Hansen GL, Seifer FD. Prevalence and incidence of noncystic fibrosis bronchiectasis among US adults in 2013. Chron Respir Dis 2017; 14:377
(3) Seitz AE, Olivier KN, Adjemian J, et al. Trends in bronchiectasis among medicare beneficiaries in the United States, 2000 to 2007. Chest 2012; 142:432
 PATOFISIOLOGI
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 Model Cole’s vicious cycle
 Respons inflamasi host
terhadap benda asing dan
bakteri di saluran napas
menyebabkan kerusakan
jaringan yang berakibat
bronkiektasis, selanjutnya
mengganggu bersihan
mukus dan terjadi
kolonisasi bakteri
6
ETIOLOGI
 Infeksi merupakan
penyebab paling umum
 Pasteur, dkk (2000) di
UK mendapatkan 53%
tidak bisa diidentifikasi
kausa spesifik
 ETIOLOGI(2)
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 Congenital : Pulmonary sequestration
 Post Infection : Tuberkulosis, whooping cough, Non-tuberculous mycobacteria (NTM)
 Immunodeficiency
 1°—common variable immune deficiency (CVID), X-linked agammaglobulinaemia (XLA,
usually diagnosed in childhood), activated PI3 kinase delta syndrome (APDS, due to rare
activating PI3Kd mutations; causes bronchiectasis which is often familial, with H. influenza and
S. pneumoniae infection; other features may include herpes virus infection, cytopenias, elevated
IgM, and lymphoma), Good syndrome (immunodeficiency with current or prior thymoma)
 2°—HIV, chronic lymphocytic leukaemia, nephrotic syndrome
 Mucociliary clearance abnormalities
 Airway diseases : Cystic fibrosis, Primary ciliary dyskinesia, COPD, Asthma, A1AT
Deficiency, Allergic bronchopulmonary aspergillosis (ABPA)
 ETIOLOGI (3)
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 Toxic insult: Aspiration, Inhalation (toxic gases, chemicals)
 Mechanical insults : Foreign body aspiration, Extrinsic lymph node compression, Intrinsic
(intraluminal) obstructing tumour
 Associated diseases: Bronchiectasis is associated with a number of systemic diseases, so
cough and sputum production in these conditions should trigger referral to determine the
cause:
 Rheumatoid arthritis (RA) (up to 35% of RA patients have bronchiectasis),
 Connective tissue diseases, e.g. Sjögren’s syndrome, systemic lupus erythematosus
 Ulcerative colitis and Crohn’s disease
 Chronic sinusitis
 Yellow nail syndrome
 Marfan’s syndrome
9 GAMBARAN KLINIS

Fishman’s Pulmonary Diseases and Disorders, 5th Edition, 2016

 DIAGNOSIS
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 Chest radiograph (CXR) sensitivity is only 50%, classically shows ‘ring shadows’ and
‘tramlines’—indicating thickened airways, and the ‘gloved finger’ appearance. Consolidation
around thickened and dilated airways. Honey comb apperance
 HRCT chest (slices <1mm, with high spatial frequency reconstruction) is 97% sensitive in
detecting disease. Typically shows airway dilatation to within 1cm of the lung periphery,
bronchial wall thickening, and the airway appearing larger than its accompanying vessel (signet
ring sign).
 Sputum microbiology Standard microscopy, culture, and sensitivity, acid-fast bacillus (AFB),
and fungal cultures
 PFTs with reversibility testing
 Obstructive Airflow
 Mixed Obstructive and restrictive
 Immunoglobulins A, M, G
 Aspergillus precipitins, Aspergillus-specific radioallergosorbent test (RAST), total IgE
 DIAGNOSIS
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 Chest radiograph (CXR) sensitivity 50%,
classically shows ‘ring shadows’ (Honey comb
appearance)and ‘tramlines’—indicating
thickened airways, and the ‘gloved finger’
appearance. Consolidation around thickened and
dilated airways.
 HRCT chest is 97% sensitive in detecting
disease. Typically shows airway dilatation to
within 1cm of the lung periphery, bronchial wall
thickening, and the airway appearing larger than
its accompanying vessel (signet ring sign).
12 TIPE MORFOLOGI
13 MANAGEMENT
 Simptomatik
 Bronkodilator bila dari pemeriksaan fungsi paru dida[at tanda obstruksi bronkus
 Oksigenasi
 Saat eksaserbasi akut: antipiretik, antibiotic,
 Mukolitik: NAC
 Fisioterapi dada untuk drainase sekret
 Pembedahan bila pengobatan tidak berhasil, biasanya pada pasien batuk darah berulang
 Pencegahan: imunisasi, pengobatan adekuat pada pasien
pneumonia/bronkopneumonia/pertussis serta morbili, menghindari paparan bahan
merangsang produksi secret, menghindari bahan iritan serta obat penekan batuk
14 STEPWISE MANAGEMENT
15 Exacerbation
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18 ANTIBIOTIC
 This may be intermittent for exacerbations only (for mild disease) or long term for more
severe disease. Antibiotics may be oral, nebulized, or IV
 Regular sputum surveillance will ensure the likely colonizing organism is known
 In vivo sensitivity may be different to in vitro sensitivity
 Patients need a higher antibiotic dose and for a longer time period (usually 10–14 d) than
people without bronchiectasis
 Antibiotic treatment choice depends on the severity of the underlying disease
 Treatment response is usually assessed by a fall in sputum volume and change to mucoid
from purulent or mucopurulent sputum, with an improvement in systemic symptoms,
spirometry, and C-reactive protein (CRP)
 Pseudomonas-colonized patients have more frequent exacerbations, worse CT scan
appearances, and a faster decline in lung function.
19 ANTIBIOTIC FOR EXACERBATION (1)
20 ANTIBIOTIC FOR EXACERBATION (2)
21 ANTIBIOTIC FOR EXACERBATION (3)
22 COMPLICATIONS

LOCAL SYSTEMIC
 Recurrent Pneumonia  Hypoproteinemia – generalisata edema
 Massive Hemoptisis  Amyloidosis-nephrotic syndrome
 secondary due to amyloidosis
Cor Pumnonale
 Respiratory Failure
 Signet-Ring Sign, Bronchiectasis. 
The bronchi (red arrows) are larger than their
corresponding arteries (white arrows), the reverse
of the normal pattern in which the bronchus is
smaller than its corresponding vessel.

23 THANK YOU